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Annals of Pathology and Laboratory Medicine July-Sept. 2015; Vol. 2, Issue 3

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Annals of Pathology and Laboratory Medicine Editorial Team Co-Editor in Chief

Dr Prashant Goyal Dr Shelly Sehgal

Associate Editor Dr Sompal Singh

Editorial Board Members

Advisory Editors

Dr Asitava Mondal Prof. Vatsala Mishra Dr Mudit Agarwal Prof. A S Ramaswamy Dr Harsh Vardhan Singh Dr Manu Noatay Dr Anil Parwani

Dr Sarah Iqbal Ch Dr Jerad M Gardner Dr Naila Atif Dr Rajan Chopra Dr Niti Singhal Dr (Prof) Severino Rey Dr Rajeshwar Reddy Dr Nasser Said-Al-Naief Dr Hoda A Hagrass Dr Kemal Turker Ulutaş Dr Dennis P O’Malley Dr Parthasarathi Pramanik Dr Arvind Rishi Dr Ahmad Mohammad Ragab Dr Amado Ona Tandoc Dr Shamim Sheikh Dr Viral M Bhanvadia Dr Navin K Sinha Dr Soumyesh Ghosh Dr Deepti Mittal Dr Awanindra Kumar Dr Manjusha Biswas Prof. Kuldeep Singh Dr Shriniwas Rushi

July-September-2015

Volume-2, No.-2

Content Original Article Ductal Carcinoma in Situ of The Breast: Modified Black Nuclear Grading System Revisited-Fernando Schuh, Jorge Villanova Biazús, José Antônio Cavalheiro, Christiane Cardoso Falcão, Alessandra de Freitas Ventura, Erika Resetkova, Diego Uchoa, Maria Isabel Edelweiss

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Cytological Features in The Early Diagnosis of Papillary Carcinoma of Thyroid in Clinically Inapparent Cases-Anshu Jain, Kiran Alam, Veena Maheshwari, Divya Rabindranath, Azka Anees Khan, Roobina Khan

A93-97

Experience of The External Quality Assessment Scheme (Eqas) in Haematology At C.u. Shah Medical College, Surendranagar-Killol Nathubhai Desai, Parth H Vithalani, Ashok S Agnihotri, Kimmyben Patel

A98-100

Case Report A Rare Renal Neoplasm with Dimorphic Histology and Mucinous Stroma-Sunitha Ramachandra, Lakshmi Rao, Masoud Al Kindi Solitary Fibrous Tumour of The Prostate with Ossification: A Rare Case Report-Neeraj Dhameja, Deepa Santosh, Vikash ., U S Dwivedi Intra-Cystic Papillary Neoplasm of Salivary Gland with Focal Low Grade Malignant Change: A Rare Entity-Sohaila Fatima, Nazima Haider, Sabah Nayef Nemri, Balkur Krishnamoortthi Adiga Cystic Partially Differentiated Nephroblastoma: Report of A Rare Case-Swagata Dowerah, Mondita Borgohain Eccrine Angiokeratomatous Hamartoma: Report of A Case with Brief Review-Vijayashree Raghavan, Devi Subbarayan, Ramesh K Rao Primary Chondrosarcoma of The Breast: A Rare Case PresentationAnshu Jain, Veena Maheshwari, Shagufta Qadri, Divya Rabindranath, Nishat Afroz Cytology: A Diagnostic Modality in Cryptococcal LymphadenitisPooja Srivastava, Kusum Gupta, Rashmi Arora Pleural Effusion Obscuring Pulmonary Echinococcosis: Diagnosis By Cytology-Anshu Jain, Veena Maheshwari, Divya Rabindranath, Kashmi Sharma

C142-145 C146-150 C151-154 C155-158 C159-162 C163-166 C167-169 C170-173

Undifferentiated Subtype of Non-Keratinizing Carcinoma of The Nasopharynx Metastasizing To The Breast: A Case Report and Literature Review-Ali Koyuncuer

C174-179

Hydatid Cyst in Femur: A Rare Case Report-Shweta Chaturvedi, Shubha Gupta, Mansi Faujdar, Gajendra Gupta, Jaimini Patel, Vishal Khandelwal

C180-182

Gigantic Lipoleiomyoma of Cervix with Extensive Hyaline Degeneration: A Case Report with Review of Literature-Rupali Mandal, Krishnendu Mondal, Pulakesh Pramanik

C183-186

July-September-2015

Volume-2, No.-2

Congenital Hepatic Fibrosis Associated with Polycystic Kidney Disease-Jaimini Natvarlal Patel, Shubha Gupta, Mansi Faujdar, Gajendra Gupta, Shweta Chaturvedi

C187-191

Low-Grade Appendicial Mucinous Neoplasm Presenting with Obstructed Inguinal Hernia: A Rare Association-Abhishek Chowdhury, Keya Basu, Uttara Chatterjee, Chhanda Datta, P.K. Sarkar, Manoj Kumar Choudhuri

C192-195

Solitary Eosinophilic Granuloma of The Radius: A Case Report-Junu Devi, Sukanya Kalita

C196-199

Splenic Lymphoma with Villous Lymphocytes; A Case Report with Review of Literature-Ruquiya Afrose, Mohammad Akram, Nirupama Panikar Khan, Usha Rusia Soft Tissue Chondromas: A Case Series of 6 Cases with Review-Ankit kaushik, Amita Malik, . Rajni, Sumit Batra, V K Sharma, Geetika Khanna

C200-203 C204-207

Letter to editor Cytopathology of Intramuscular Myxoma-Shilpi Agarwal, Shivali Sehgal, Preeti Rai, Priya Thomas Tumor or Gossypiboma-Pankaj Gupta, Shruti Chaudhary, Noopur Gupta, Sanjay Deb, Sumedha Kotwal, Ramesh Dawar An Umbilcal Polyp in an Infant: A Rare Congenital Lesion-Meghna Singh, Jitendra Chaudhary Spindle Cell Lipoma: An Unusual Lipoma Variant-Pranshuta Sharma, Neelam Sood

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Original Article Ductal Carcinoma In Situ of The Breast: Modified Black Nuclear Grading System Revisited Fernando Schuh1*, Jorge Villanova Biazús1, José Antônio Cavalheiro1, Christiane Cardoso Falcão, Alessandra de Freitas Ventura2, Erika Resetkova3, Diego Uchoa4, Maria Isabel Edelweiss4 1

Breast Surgeon, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Brazil 2 Breast Surgeon, Hospital São Cristóvão, Brazil 3 Department of Pathology, The University of Texas MD Anderson Cancer Center, USA 4 Department of Pathology, Hospital de Clínicas de Porto Alegre, Brazil Keywords: Classification, ductal carcinoma in situ, scoring system, interobserver reproducibility.

ABSTRACT Background: This study aims to determine the pathologists’ agreement of modified Black nuclear grading system and Holland classification applied to cases of ductal carcinoma in situ (DCIS). Methods: Forty-three cases of breast lesions diagnosed as DCIS were selected to interobserver analysis. Eight pathologists received the same set of digitized images from microscopy of DCIS cases, and answered a questionnaire containing the criteria to compose the modified Black nuclear grade and Holland classification. In order to determine interobserver agreement and diagnostic accuracy, a web-based survey was created. It organizes the information collected from each participant pathologist providing the histological grading of the cases in both classification systems. Result: Comparing the two classifications studied, there was a similar interobserver agreement among both schemes, showing Kappa value of 0.28 ± 0.02 for the modified Black nuclear grade and 0.32 ± 0.02 for the Holland classification. Hence the reliability for the applied to cases of DCIS was considered acceptable. The agreement among all pathologists and the gold standard pathologist similarly followed the results of the interobserver agreement, showing to be acceptable, with Kappa for de overall mode value 0.33 + 0.10 for modified Black nuclear grade and 0.55 + 0.10 for Holland classification (p = 0.07). The findings of Kappa for the mode values among specialists in breast pathology and general pathologists were, respectively, 0.34 + 0.11 (acceptable) and 0.26 + 0.10 (acceptable) for the modified Black nuclear grade and 0.50 + 0.10 (acceptable) and 0.26 + 0.10 (acceptable) for Holland classification. Breast pathology specialists showed similar reproducibility for both evaluated classifications than pathologists not devoted to this subject. Conclusion: The diagnostic accuracy was similar for modified Black nuclear grading system regarding Holland classification system.

*Corresponding author: Fernando Schuh, Rua Duque de Caxias 1191/304, Porto Alegre, Brazil Phone: +91-55 51 3234 6137 E-mail: fernandoschuh@hotmail.com

This work is licensed under the Creative Commons Attribution 4.0 License. Published by Pacific Group of e-Journals (PaGe)

Schuh et al.

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Introduction

characteristics and patterns of necrosis were used to compose the different classification systems12,20, 24,25.

The introduction of mammographic screening in the 1980s generated an explosive increase in the incidence of ductal carcinoma in situ (DCIS) by more than 500% from 1983 to 1992. Currently it represents approximately 30–40% of all mammographically detected breast cancers. Consequently this has led to an increased interest in the biology, classification, clinical behaviour, and treatment of DCIS1,2,3,4 .

The classification of Holland24, used by the European Pathologists Working Group, emphasizes primarily cytonuclear differentiation and secondarily architectural differentiation (cellular polarization). This system classifies DCIS in three groups: poorly, moderately (intermediately), and well differentiated24. Black and colleagues26,27 evaluated the prognostic significance of the tubule formation and the nuclear features separately and concluded that only nuclear morphology is a significant prognostic factor. They proposed a nuclear grading system with five grades. Contrary to common practice, grade 0 and 1 were used to designate the most poorly differentiated, or anaplastic neoplasms, whereas grade 4 reflected the well-differentiated tumors. This reversal of the numerical order remained a disturbing aspect of this nuclear grading system and contributed to a lack of wide support for its application. The nucleargrading system of the Black and colleagues has been found to be useful in predicting prognosis28.

It has been estimated that if left untreated, DCIS will develop to invasive carcinoma in a significant proportion of cases, generally within 10 years of diagnosis. Clinical trials showed beneficial effect of lumpectomy especially with adjuvant radiation in cases of DCIS5,6,7,8. In some instances, DCIS may recur locally, and 50% recurs as invasive carcinomas9,10,11. Clearly, the most important determinant of recurrence is the adequacy of surgical excision, but pathological assessment of excision margins is beset with technical difficulties12,13. The DCIS is strictly defined as a proliferation of epithelial cells with malignant cytological and histological features within the terminal duct-lobular unit of the breast, confined within the basement membrane. DCIS is not a single morphological entity but a heterogeneous group of proliferative breast lesions with different malignant potentials that varies according to cytology and growth pattern. This reflects its clinical presentation, histopathology, radiologic features, expression of biological markers, and clinical behaviour9,10,11. DCIS classification has traditionally been based on growth pattern; a number of studies have shown a relation between this aspect and behaviour. Growth pattern, however, often varies from one part of the tumour to another, which at least partly explains why architectural classifications are associated with a low level of observer consistency[11-16].

Fisher and coworkers29 devised a grading method and modified the Black nuclear grading system by reducing it from five to three grades after combining grades 0 and 1 into one group, and grades 3 and 4 into another. Furthermore, they inverted the numerical order so that grade 1 corresponded to the well differentiated carcinomas and grade 3 reflected the most poorly differentiated ones. There is no report in literature that has explored the issue of interobserver reproducibility and diagnostic accuracy according to the modified Black nuclear grading system. Table 1 shows the morphologic criteria for the two classification systems studied, as well as their graduation. This study was performed to assess agreement, comparing interobserver results, and to determine the accuracy of the histological grade of modified Black nuclear grading system29 and Holland classification24 for DCIS, using a webbased program developed to facilitate the classification. Based on these findings, we identified, among the systems

Sub categorization of the ductal proliferations is far more difficult and has resulted in persistent interobserver variability among experienced pathologists, even when the same criteria are applied17-23. Cytonuclear, cytoarchitectural

TABLE 1: Morphologic criteria for each classification system analyzed in this study. System

Nuclear grade

Cell polarization

Final DCIS grade

Holland (HL)

Well differentiated

Prominent

• Well differentiated

Intermediately differentiated

Present, not prominent

• Intermediately differentiated

Poorly differentiated

Absent/very focal

• Poorly differentiated

N/A

• Grade 1 (low grade)

Modified Blacks Nuclear Grade (MBNG)

• Grade 2 (intermediate grade)

• Grade 3 (high grade)

Note: N/A, no available

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studied, the one with the highest degree of agreement and reliability. The factor of study was the analysis of DCIS classification systems comparing modified Black nuclear grading system13 with Holland classification25. The outcome was the degree of interobserver agreement.

Materials and Methods Slides of 43 cases of DCIS of the breast, diagnosed at “Hospital de Clínicas de Porto Alegre” (HCPA), Brazil, and at MD Anderson Cancer Center, USA, were chosen by convenience sampling. Typical examples of DCIS were considered to select these cases, as well as those where histological material was well processed. The slides selected were reviewed by two experts in breast pathology without knowledge of the clinical and demographic characteristics of patients. The cases were not selected based on ease of diagnosis, but because they represented different grades of tumor differentiation. Cases in which there was evident invasive ductal carcinoma associated or divergence between the original anatomopathological diagnosis and the review performed at selection were excluded. The case slides were stored at the HCPA Pathology Service and in the Pathology Department of University of Texas MD Anderson Cancer Center, and they were prepared from surgical specimens fixed in buffered formalin and placed in paraffin blocks, using 5µm thick sections stained with hematoxylin-eosin. The cases were presented in digital photographs, in JPEG format, to the pathologists participating in this study. The reviewing pathologists obtained several colored digital photomicrographs of the selected DCIS cases. The website created for this study provides images of the same field in three different magnifications (100, 200, and 400×). During analysis, the pathologist has the opportunity to enlarge each image provided. Each case had at least 5 images stored in JPG format, which the observers could access freely during the assessment with or without magnification. A cross-sectional study was carried out to diagnose the grading of DCIS cases in order to assess the degree of agreement between pathologists in the city of Porto Alegre, Brazil. The privacy of all data obtained was ensured, and this information was used exclusively for the scientific purpose expressed in this research project, with a guarantee that the identity of participants would be kept confidential. This project was approved by the Ethics and Research Committee of the Graduate and Research Group (GPPG) at “Hospital de Clínicas de Porto Alegre”.

Participating Pathologists

Pathologists who work in Porto Alegre hospitals with breast cancer services were invited to participate in this study. Among 35 pathologists invited by the researchers, 12 agreed to the terms of the letter of free and informed

consent to participate in the study: 4 university lecturers, 4 medical assistants, and 4 medical residents. Three of them said that they were specialists in breast pathology. The invited participants, except for the medical residents, had the title of specialist in pathology, and were associated members of the Brazilian Society of Pathology. The medical residents were excluded for statistical analysis. All of them work at hospitals connected to centers of academic training. A pathologist specializing in breast pathology from the MD Anderson Cancer Center, USA (ER) was invited to act as gold standard.

Statistical Analysis The Kappa statistical method was used to access interobserver variability and agreement of invited pathologists with the expert pathologist for diagnosis in each classification system. The Kappa (κ) values vary from 0 to 1, and 1 indicates perfect agreement. Lands and Koch suggest the following interpretation for different ranges of Κappa values: from 0 to 0.20 agreement equals poor or weak; from 0.21 to 0.40 agreement is acceptable; from 0.41 to 0.60 equals moderate; from 0.61 to 0.80 equals good; and from 0.81 to 1.00 equals excellent30. To evaluate the diagnostic agreement between pathologists, the value of κ was estimated for multiple categories and multiple observers. Statistical comparison of agreement between classifications was done using a comparison test between Kappa values for subgroups of pathologists using the formula from Svanholm et al.31. For each classification, the proportion of cases in which most pathologists agreed with the expert (gold standard) was also estimated. Moreover, the subgroups of pathologists were also evaluated by the mode value. Whenever there was a draw, it was assumed the higher grade one as the mode value. In order to estimate diagnostic accuracy, the value of Κappa was calculated for each pathologist and for the pathologist subgroups mode values. Program SPSS v.14.0 was used for statistical analysis of the data. According to the calculation, for a 0.7 Kappa, 95% confidence interval and 15% margin of error, at least 23 different cases of DCIS would be needed.

Result None of the participants (experts and non-specialists) disagreed with the diagnosis of DCIS in the 43 cases evaluated. The eight pathologists perform their professional activities in hospitals linked to academic training centers. Three of the pathologists are experts in breast pathology. Five pathologists mentioned that they generally employ Van Nuys classification to grade intraductal lesions of the breast and one pathologist adopts Holland classification. All

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participants confirmed that they had practiced pathology for more than 5 years. The expert pathologist defined as gold standard reports using modified Black classification for nuclear grade and Van Nuys in her routine to classify DCIS, and had practiced pathology between 7 and 15 years. Table 2 shows the proportion of cases diagnosed for the two different classification schemes by the pathologist gold standard (the expert in breast pathology from the MD Anderson Cancer Center, USA). Table 3 shows the proportion of diagnosed cases according to Holland classification and modified Black nuclear grading system by each of the 8 pathologists, separated by subgroups: breast pathology specialists and not experts in breast pathology. The classification systems evaluated in this study presented acceptable interobserver agreement, as shown in Table 4. The classification systems of DCIS of the breast showed a similar overall diagnostic agreement and obtained Kappa index of 0.28 + 0.02 for Black and 0.32 + 0.02 for Holland.

Table 5 shows the degree of agreement between the pathologists and the gold standard pathologist (accuracy) for the two classification systems covered in this study. For modified Black nuclear grading system, there was agreement among the 8 pathologists and the gold standard in 9 cases (20.93%). Taking experts into consideration, there was complete concordance with the gold standard in 20 cases (46.51%), 18 of those corresponding to highgrade DCIS and two cases corresponding to low and moderate. The accuracy for each pathologist was estimated with Kappa statistics, and ranged from 0.02 + 0.08 to 0.63 + 0.10, considered weak and good, respectively. The finding of Kappa values for the mode value representing all participating pathologists was 0.33 + 0.10. For the experts in breast diseases Kappa values ranged from 0.18 + 0.10 to 0.63 + 0.10 (weak and good, respectively), and for the no specialists, from 0.02 to 0.49 (weak and moderate, respectively). Using the mode value, the Kappa value for experts was 0.34 + 0.11(acceptable), while for no specialists it was 0.26 + 0.10 (acceptable).

TABLE 2: DCIS cases classified in each histological grade in the two classification systems, by the gold standard reference pathologist. Grade3 Grade 2 Grade 1 Poorly differentiated Intermediately differentiated Well differentiated n (%) n (%) n (%) Modified Blacks Nuclear Grading System (MBNGS) 21 (48.8) 10 (23.3) 12 (27.9) Holland (HL)

24 (55.8)

10 (23.3)

9 (20.9)

TABLE 3: Distribution of histological grading scores of 43 DCIS cases evaluated by the 8 pathologists. Modified Black Nuclear Grade Type

Pathologist

Grade 3 n (%)

Grade 2 n (%)

Grade 1 n (%)

Pathologists specialized in breast disease

A B C D E F G H

27 (62.8) 28 (65.1) 23 (53.5) 27 (62.8) 17 (39.5) 33 (76.7) 20 (46.5) 27 (62.8)

13 (30.2) 13 (30.2) 13 (30.2) 8 (18.6) 17 (39.5) 8 (18.6) 22 (51.2) 15 (34.9)

3 (7.0) 2 (4.7) 7 (16.3) 8 (18.6) 9 (21.0) 2 (4.7) 1 (2.3) 1 (2.3)

Pathologists no specialized in breast disease

Holland Interm. Poorly differ. differ. n (%) n (%) 22 (51.2) 19 (44.1) 33 (76.7) 6 (14.0) 25 (58.0) 9 (21.0) 25 (58.1) 11 (25.6) 21 (48.8) 14 (32.6) 27 (62.8) 14 (32.6) 18 (41.9) 24 (55.8) 20 (46.5) 23 (53.5)

Well differ. n (%) 2 (4.7) 4 (9.3) 9 (21.0) 7 (16.3) 8 (18.6) 2 (4.7) 1 (2.3) 0 (0.0)

TABLE 4: Degree of interobserver agreement of breast CDIS for the two systems studied.

Modified Black Nuclear Grading System (MBNGS)

All participating pathologists n=8 k + SE 0.28 Âą 0.02

Pathologists specialized in breast disease n=3 k + SE 0.43 Âą 0.07

0.32 + 0.02

0.38 + 0.09

Holland (HL)

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TABLE 5: Degree of agreement of the pathologists with the expert pathologist (gold standard) for the two classification systems studied Modified Black Holland Type Pathologists k ± EP k ± EP A 0.38 ± 0.10 0.50 ± 0.10 B 0.18 ± 0.10 0.37 ± 0.11 Pathologists specialized in breast disease C 0.63 ± 0.10 0.64 ± 0.10 MODE 0.34 ± 0.11 0.50 ± 0.10 D 0.49 ± 0.11 0.40 ± 0.11 E 0.40 ± 0.11 0.47 ± 0.11 F 0.02 ± 0.08 0.30 ± 0.10 Pathologists no specialized in breast disease G 0.21 ± 0.10 0.19 ± 0.10 H 0.27 ± 0.10 0.24 ± 0.10 MODE 0.26 ± 0.10 0.26 ± 0.10 Total MODE 0.33 ± 0.10 0.55 ± 0.10

p-values: 0.454 when comparing the mode for specialists with no specialists for MBNGS. 0.053 when comparing the mode for specialists with no specialists for HL. 0,070 when comparing MBNGS and HL for all groups of pathologists. Considering the Holland classification, there was a complete agreement among the 8 pathologists and the expert in 8 cases (18.6%). The observed agreement between the pathologists and the gold standard pathologist had the test Kappa index ranging from 0.19 + 0.10 to 0.64 + 0.10, considered weak and good, respectively, corresponding to the Kappa index of 0.55 + 0.10 for the mode value. As noted, the accuracy was better in the expert pathologists group, Kappa values ranged from 0.37 + 0.11 to 0.64 + 0.10, values considered acceptable and good, respectively. For the mode value, the experts had a Kappa index of 0.50 + 0.10 (acceptable). In this group, there was complete concordance with the gold standard in 23 cases (53.49%), comprising 19 cases of DCIS poorly differentiated, 2 cases of intermediately differentiated and 2 cases of well differentiated. Comparing the modified Black nuclear grade and the Holland classification there was no statistical significance difference between the Kappa of the mode for all groups of pathologists (p = 0.070).

Discussion Several studies have examined the degree of agreement in DCIS classifications17,21,23,32. All of them varied in details, such as number of cases, origin of cases, divergence in the diagnosis of difficult cases, mode of exhibition of the cases, association with invading component, number of readings performed, pathologists origin and experience, and also whether there was some form of training prior to evaluating the cases. All the classification systems proposed consider primarily nuclear grade and necrosis, leaving the architectural pattern aside, or not taking it into account at all. The terminology of the classifications and the criteria used to compose them are different, but they

share the recognition of three main subtypes of DCIS: high, intermediate, and low grade4,11. An ideal classification for DCIS should present a sort of characteristics. Firstly, it should be clinically useful, correlating the histological grade with the rate of local recurrence and progression to invasive carcinoma. Secondly, it should provide precise and unambiguous terms to define the characteristics of all different types of DCIS. Furthermore, it should be simple and easy to apply, even in cases with minimal DCIS compromised ducts. A high rate of agreement among pathologists is also important7. This study included 43 cases of DCIS selected by convenience sampling. The data analyzed were obtained through a standardized questionnaire on Internet containing the criteria to classify the cases of DCIS of the breast. The use of a computer program, created in our institution, makes the process of classification simpler and faster, since the program itself determines the final grade in each system studied. This model was also adopted to restrain the subjective description of the histopathological criteria evaluated. Likewise, the cases were presented in digitized images, allowing every pathologist to evaluate the same area of interest of the histological section in each case. Even using this method that provides a more objective analysis, it was observed that the reproducibility remained low for the systems evaluated. Moreover, the use of digitized images may have impaired the evaluation of the number of mitoses, due to the lack of microscopic fields, so the pathologist had to estimate this information for each case. As the modified Black nuclear grade take into account the number of mitoses, it could have contributed to low degree of agreement shown in our study.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Schuh et al. The comprehensive evaluation of five DCIS classifications by 23 European pathologists (the European Commission Working Group on Breast Screening Pathology) found that the inclusion of cell polarization, besides the nuclear grade, in reaching a final DCIS grade using Holland classification neither improved nor worsened the level of consistency that could be achieved by using nuclear grade only21. In the current study, the reproducibility of Holland classification and modified Black nuclear grading were similar, confirming that previous affirmative. However, there is still no evidence that any characteristic beyond nuclear grade and necrosis has prognostic significance. Our study showed low interobserver agreement on the final histological grade for the Holland and modified Black nuclear grading system. Whenever participants were divided into subgroups according to their interest in breast pathology, greater agreement was found among those selfnamed breast specialists. This fact suggests that probably the criterion that has greater influence on pathologists’ agreement is the appropriate training and the development of precise definitions.

A-91 microscopic features of the lesions in question without the aid of surrounding histological cues. Regardless, our results should be analyzed with some caution, because this is a study with a unique methodology, both in case presentations and in the determination of the histological grade, based on criteria of a standardized questionnaire.

Acknowledgements The authors declare no competing interests.

Funding This research was funded by Hospital de Clínicas de Porto Alegre

Competing Interests Competing interests: None declared

Reference

There are a number of potential limitations in this study. First, it could be argued that our results may not be representative of the level of agreement attainable in general pathology practice because of the small numbers of participating pathologists. We must also consider the fact that the participants were volunteers who made themselves available to participate in this study. Second, the pathologists in this study were asked to render their diagnosis following examination of selected digital images rather than following examination of whole histological sections under the microscope, as it is done in routine clinical practice. However, given that the goal of this study was to assess observer agreement in the classification of specific lesions, we believe that the use of digital images could be regarded as a strength of the study as it required the participants to base their diagnosis solely on

1. Ernster VL, Ballard-Barbash R, Barlow WE, et al.: Detection of ductal carcinoma in situ in women undergoing screening mammography. J Natl Cancer Inst 2002, 94:1546–54. 2. Ernster VL, Barclay J, Kerlikowske K, et al.: Incidence of and treatment for ductal carcinoma in situ of the breast. JAMA 1996, 275:913–8. 3. Kerlikowske, K: Epidemiology of Ductal Carcinoma In Situ. J Natl Cancer Inst Monogr 2010, 41:139-141. 4. Tavassoli FA, Devilee P (Eds): World Health Organization Classification of Tumours. Pathology and genetics of tumours of the breast and female genital organs. IARC Press: Lyon 2003, 60-81. 5. Fisher B, Dignam J, Wolmark N, et al.: Lumpectomy and radiation therapy for the treatment of intraductal breast cancer: findings from National Surgical Adjuvant Breast and Bowel Project B-17. Journal of Clinical Oncology 1998, 16: 441-452. 6. Fisher ER, Costantino J, Fisher B, et al.: Pathologic findings from the National Surgical Adjuvant Breast Project (NSABP) protocol B-17: Intraductal carcinoma (ductal carcinoma in situ). Cancer 1995, 75:1310-19. 7. Julien JP, Bijker N, Fentiman IS, et al.: Radiotherapy in breast-conserving treatment for ductal carcinoma in situ: first results of the EORTC randomised phase III trial. EORTC Breast Cancer Cooperative Group and EORTC Radiotherapy Group. Lancet 2000, 355:528-33. 8. Kane RL, Virnig BA, Shamliyan T, et al.: The Impact of Surgery, Radiation, and Systemic Treatment on Outcomes in Patients With Ductal Carcinoma In Situ. J Natl Cancer Inst Monogr 2010, 41:130-133.

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The most important aspect that can be drawn from the current study is that the modified Black nuclear grading system showed the same interobserver reproducibility and accuracy than the classification of Holland for DCIS. Therefore, the modified Black nuclear system can be an alternative to classify DCIS breast lesions. Pathologists specialized in breast pathology showed greater reproducibility and accuracy for both classifications compared to the others subgroups. These findings lead us to conclude that the academic institutions of this city should introduce training and quality-control programs in order to improve the rates of diagnostic agreement.

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9. Burstein HJ, Polyak K, Wong JS, et al.: Ductal carcinoma in situ of the breast. N Engl J Med 2004, 350:1430-41.

21.

10. Harris JR, Lippman ME, Morrow M, Osborne CK: Diseases of the breast. Lippincott Williams e Wilkins; 2004. 11. Rosen PP, Oberman H: Tumors of the mammary gland. Armed Forces Institute of Pathology; 1993. 12. Lagios MD, Margolin FR, Westdahl PR, et al.: Mammographically detected duct carcinoma in situ. Frequency of local recurrence following tylectomy and prognostic effect of nuclear grade on local recurrence. Cancer 1989, 63: 618-624. 13. Schwartz GF, Finkel GC, Garcia JC, et al.: Subclinical ductal carcinoma in situ of the breast. Treatment by local excision and surveillance alone. Cancer 1992, 70:2468-74. 14. Badve S, A’Hern RP, Ward AM, et al.: Prediction of local recurrence of ductal carcinoma in situ of the breast using five histological classifications: a comparative study with long follow-up. Hum Pathol 1998, 29:915-23.

22.

23.

24.

25.

26.

15. Goldhirsch A, Glick JH, Gelber AS, et al.: Meeting Highlights: International expert consensus on the primary therapy of Early Breast Cancer 2005. Annals of Oncology 2005, 16:1569-83.

27.

16. Page DL, Dupont WD, Rogers LW, et al.: Intraductal carcinoma of breast: Follow up after biopsy only. Cancer 1982, 49:751-758.

28.

17. Bethwaite P, Smith N, Delahunt B, et al.: Reproducibility of a new classification schemes for pathology of ductal carcinoma in situ of the breast. J Clin Pathol 1998, 51:450-4.

29. 30.

18. Douglas-Jones AG, Morgan JM, Appleton MA, et al.: Consistency in the observation of feature used to classify duct carcinoma in situ (DCIS) of the breast. J Clin Pathol 2000, 53:596-602.

31.

19. Salles MA, Mattos MA, Resende IM, et al.: Análise interobservador no diagnóstico histopatológico do carcinoma ductal in situ da mama. Rev Brasil Ginecol Obstet 2005, 27:1-6.

32.

20. Scott MA, Lagios MD, Axelsson K, et al.: Ductal Carcinoma in situ of the Breast: Reproducibility of

Histological Subtype Analysis. Hum Pathol 1997, 28:967-73. Sloane JP, Amendoeira I, Apostolikas N, et al.: Consistency achieved by 23 European pathologists in categorizing ductal carcinoma in situ of the breast using five classifications. Hum Pathol 1998, 10:105662. Sneige N, Lagios MD, Schwarting R, et al. Interobserver reproducubility of the Lagios nuclear grading system for ductal carcinoma in situ. Hum Pathol 1999, 30:257-62. Wells WA, Carney PA, Eliassen MS, et al.: Pathologists’ agreement with experts and reproducibility of breast ductal carcinoma in situ classification schemes. Am J Surg Pathol 2000, 24:651-9. Holland R, Peterse JL, Millis RR, et al.: Ductal carcinoma in situ: A proposal for a new classification. Semin Diagn Pathol 1994, 11:167-70. Silverstein MJ, Lagios MD, Craig PH, et al.: A prognostic index for ductal carcinoma in situ of the breast. Cancer 1996, 77:2226-2274. Black MM, Barclay TH, Hankey BF, et al.: Prognosis in breast cancer utilizing histologic characteristics of the primary tumor. Cancer 1975, 36:2048-55. Black MM, Opler SR, Speer FD: Survival in breast cancer cases in relation to the structure of the primary tumor and regional lymph nodes. Surgery Gynecology & Obstetrics 1995, 100:543-51. Tavassoli FA: Patology of the Breast. McGraw-Hill, 1999. Fisher ER, Redmond C, Fisher B: Histologic grading of breast cancer. Pathol Annu 1980, 15:239-51. Landis JR, Koch GG: The measurement of observer agreement for categorical data. Biometrics 1977;33:159-74. Svanholm H, Staklint H, Gundersen HJG, et al.: Reproducibility of histomorphologic diagnosis with special reference to the kappa statistic. APMIS 1989, 97:689-698. Douglas-Jones AG, Gupta SK, Attanoos RL, et al.: A critical appraisal of six modern classifications of ductal carcinoma in situ of the breast (DCIS): correlation with grade of associate invasive carcinoma. Histopathology 1996, 29:397-409.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Original Article Cytological Features in The Early Diagnosis Of Papillary Carcinoma Of Thyroid In Clinically Inapparent Cases Anshu Jain, Kiran Alam, Veena Maheshwari, Divya Rabindranath*, Azka Anees Khan, Roobina Khan Department of Pathology, J.N. Medical College, A.M.U. Aligarh, India Keywords: Papillary Thyroid Carcinoma, Cytology, Papillary Clusters, Nuclear Grooves

ABSTRACT Background: Papillary thyroid carcinoma (PTC) is the most common among the thyroid malignancies. Fine needle aspiration cytology (FNAC) is the first line investigation in the diagnosis of thyroid lesions. Here we review the significance of certain features conventionally considered to be diagnostic of papillary thyroid carcinoma on FNAC â&#x20AC;&#x201C; including type of background, type of colloid, presence or absence of papillary clusters, intranuclear inclusions and nuclear grooves which were also considered for their importance in early diagnosis of PTC. Methods: We reviewed all the thyroid FNACs performed over 18 months period and correlated them clinically and histopathologically, wherever available. Results: Out of a total of 354 cases of thyroid FNACs, histopathological correlation was possible in 90 cases, with a concordant diagnosis in 82 cases. Analysis of the 8 discordant FNAC smears revealed papillary clusters along with nuclear features, especially nuclear grooves to be the most important finding in indicating a diagnosis of PTC. Conclusion: Although the presence of papillary clusters along with characteristic nuclear features on FNAC is well known to indicate diagnosis of PTC, these findings can be easily missed on cytology smears, especially when the lesions are small (less than 1 cm) and diagnosis is clinically inapparent. Hence, we propose that the presence of papillary clusters in any case should alert the pathologist towards a diagnosis of PTC. A diligent search for nuclear features should follow. Thus a high index of suspicion while examining the FNAC smears in these patients will help in early detection of PTC.

*Corresponding author: Dr. Divya Rabindranath, Department of Pathology, J.N. Medical College, A.M.U., Aligarh-202002. Uttar Pradesh, India. Phone: +91-7895683197 E-mail: divy30@hotmail.com

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Cytology of clinically inapparent thyroid papillary carcinoma

Introduction

Nodular tumors are the most common pathologies of the thyroid and they have been seen in up to 60 % of the population.[1,2] As we know, papillary thyroid carcinoma (PTC) has been shown to be the most common malignant tumor of the thyroid.[3] The pathological diagnosis and classification of PTC rests on the microscopic features of the specimens. Fine needle aspiration (FNA) of the thyroid has now become a well-established method for initial evaluation of thyroid tumors, because of its high level of accuracy in predicting PTC (reaching up to 90%). [4] However, the final diagnosis of benign or malignant nature of the tumor is based on the histopathological features of the tumor following resection. Histological and cytological diagnosis of PTC has always been one of the most problematic dilemmas in surgical pathology. There are limited studies that specifically address details of cytologic features associated with cytohistologic discrepancy. Although cytomorphological features associated with PTC have been well defined by various authors (including papillary fronds, monolayered sheets, tissue fragments, intranuclear cytoplasmic inclusions, type of colloid, psammoma bodies, and multinucleated giant cells), none of the features alone is considered diagnostic for PTC. Various studies have shown that PTC is often underdiagnosed or overdiagnosed.[5]

Materials and Methods

This was a retrospective study which included a total of 354 patients who underwent thyroid FNAC in the cytopathology laboratory of our institute over duration of 18 months. In each case, FNAC had been done with the help of a 23-gauge needle on a10ml syringe attached to a pistol-type handle. Smears had been prepared and immediately fixed in 90 % ethanol, and further stained with Papanicolaou and hematoxylin and eosin stains. 90 of the 354 cases had subsequently undergone surgery, and histological specimens were available in these cases. FNAC smears of all these patients were reviewed and correlated clinically and histopathologically, with special emphasis on cases showing histopathological diagnosis of PTC and discordant FNAC findings.

We aim to address the reliability and significance of certain cytological features in the diagnosis of PTC. These features are; Type of background, Type of colloid, Papillary fragments, Intranuclear inclusions, and Nuclear grooves. We also suggest certain cytological features which would be helpful in the early diagnosis of PTC, i.e, diagnosing lesions that are less than 1 cm in size and not apparent clinically.

Result

Among 90 cases of thyroid cytology in which histopathological correlation was possible, eight had been diagnosed as PTC on histopathology, while they had been missed on cytology. We reviewed these eight cases in detail and found that most of them were females (7 of 8, 87.5%) younger than 35 years of age (6 of 8, 75%), and presented as a case of multinodular goiter (7 of 8, 87.5%). PTC was not suspected clinically in any of these cases. Six cases were initially put in the category of non neoplastic thyroid lesions on cytology while two were labeled as unsatisfactory. On histopathological review, the diagnosis was changed to PTC in all eight cases with seven being the routine variant and one, the follicular variant of PTC. It is important to note here that most of these cases (6 of 8, 75%) were small lesions, with a maximum diameter of less than 1 cm. The cytology slides of these eight cases were further studied in detail once more, especially noting five important features, i.e.: Background, Colloid, Papillary clusters, Nuclear grooves, and Intranuclear inclusion. On studying the background, we noted that six of the cases (75%) had abundant normal epithelium in the background while two (25%) had scant cellularity in background (Fig.1). The colloid was scant in five of the eight cases (62.5%) while it was moderate in three cases (37.5%) (Fig.2). Papillary clusters proved to be an important criterion, noted in seven of the eight cases (87.5%) (Fig.3). Nuclear grooves were seen in five of the eight cases (62.5%) (Fig.4), while intranuclear inclusions were seen in only three cases (37.5 %) (Fig.5).

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Fig. 1: Unsatisfactory smear with scant colloid and single cluster of cells with papillaroid arrangement (Pap, 4x)

Fig. 2: Smear showing papillary fragment of follicular cells with thick colloid (arrow) (H&E, 10x)

Fig. 3: Follicular cells arranged in papillary architecture (H&E, 10x)

Fig. 4: Smear showing foamy cells in papillary carcinoma with nuclear grooves

Fig. 5: Smear showing intranuclear inclusions (H&E, 40x)

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Cytology of clinically inapparent thyroid papillary carcinoma

Table 1 describes the initial FNAC diagnosis, histopathology findings and findings on review of cytology in the eight discordant cases. Table 1: Initial FNAC diagnosis, histopathological findings and findings on review of FNAC smears in eight discordant cases FINDINGS ON REVIEW OF FNAC SMEARS Abundant normal epithelium

BACKGROUND

Scant

No. of cases

COLLOID

Scant

Moderate, bubble gum type

No. of cases

PAPILLARY CLUSTERS

Present

Absent

No. of cases

NUCLEAR GROOVES

Present

Absent

No. of cases

INTRANUCLEAR INCLUSIONS

Present

Absent

No. of cases

Discussion

The management of thyroid nodules relies on the clinical presentation of the patient, tumor characteristics, and investigation findings, all of which must be considered when making a decision about the treatment approach. It is imperative to correlate the clinical context with radiology findings and, most importantly, findings on FNAB. While additional investigations such as immunostaining may be useful in the future; they do not play a major role in routine diagnostic process at present. The basic question the physician wants answered is whether the thyroid nodule is benign or malignant, to define the further management of the patient- active intervention or careful observation. Fine-needle aspiration biopsy is the most helpful diagnostic method and is now considered essential in the initial workup of any thyroid nodule. The cytological appearance of PTC has been well defined by different authors. Kini et al,[6] described six parameters for PTC: Papillary tissue fragments, monolayer sheets of follicular cells, intranuclear cytoplasmic inclusions, nuclear grooves, tissue fragments with or without a follicular pattern, and large multinucleated foreign body-type of giant cells in the absence of degenerative changes.

According to Wu et al,[7] the most frequent cytology findings in PTC were flat syncytial sheets, nuclear enlargement, fine chromatin, nuclear grooves, intranuclear cytoplasmic inclusions, and some amount of colloid. Castro-Gomez et al,[8] described 15 features of PTC in FNA smears of thyroid: Tridimensional fragments, papillae, anisonucleosis, nuclear bars (grooves), intranuclear cytoplasmic inclusions, powdery chromatin, vacuolated cytoplasm, metaplastic cytoplasm, psammoma bodies, autolysis, multinucleated giant cells, spindle cells, colloid, monolayer lamina, and macrophages. Kumar et al,[9] suggested cellular swirls in cytology smears are highly specific for PTC. Despite these well-defined cytology features, diagnosis of PTC on cytology is often difficult and currently, no international standard exists for the cytological diagnosis of PTC. Recently, extensive studies have been done on these cytological criteria of PTC and their usefulness has been significantly debated. Christine R. Fraser et al described 3 cases with hypercellularity and papillary fragments on FNAB, suggesting a diagnosis of PTC but which were later found to be benign on histology. [10] In our study, we found that 87.5% of the cases of PTC showed papillary clusters. Gould et al. found in their study that 100% of the papillary carcinomas contained nuclear grooves while only 70% contained inclusions. Grooves, however, could be seen in 70% of nonpapillary neoplasms and in 56% of nonneoplastic conditions of the thyroid also. Inclusions were present in 13% of nonpapillary neoplasms and were absent in nonneoplastic conditions. [11] In comparison, we found nuclear grooves and intranuclear inclusions in 62.5% and 37.5% of the cases of PTC respectively. Psammoma bodies can also be a confusing feature as it can be found in PTC as well as many benign conditions like multinodular goiter. Erin Ellison et al found in their study that psammoma bodies were as predictive of multinodular goiter as PTC. [12] Keeping in mind the previous published reports on this topic combined with our own findings in this study, we have found papillary clusters along with nuclear features especially nuclear grooves, to be the most important finding in indicating a diagnosis of

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Rabindranath et al.

PTC. These features should alert the pathologist to the possibility of a diagnosis of papillary carcinoma of the thyroid, , even in cases which are not primarily suspected to have PTC clinically. However, none of these features are diagnostic on their own and a thorough study of all the cytological features and their correlation is necessary in each case.

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2. Gharib H, Papini E, Paschke R. Thyroid nodules: a review of current guidelines, practices, and prospects. Eur J Endocrinol.2008;159:493-505.

3. Carcangiu ML, Zampi G, Pupi A, Castagnoli A, Rosai J. Papillary carcinoma of the thyroid- A clinicopathologic study of 241 cases treated at the University of Florence, Italy. Cancer. 1985;55:805-28. 4. Renshaw AA. Accuracy of thyroid fine-needle aspiration using receiver operator characteristic curves. Am J Clin Pathol. 2001; 116: 477-82. 5. Mahajan A, Lin X, Nayar R. Thyroid Bethesda reporting category, suspicious for papillary thyroid carcinoma, pitfalls and clues to optimize the use of this category. Cytopathology.2013; 24:85-91. 6. Kini SR, Miller JM, Hamburger JI, Smith MJ. Cytopathology of papillary carcinoma of the thyroid by fine needle aspiration. Acta Cytol.1980; 24:511-21. 7. Wu HH, Jones JN, Grzybicki DM, Elsheikh TM. Sensitive cytologic criteria for the identification of follicular variant of papillary thyroid carcinoma in fineneedle aspiration biopsy. Diagn Cytopathol.2003;29: 262-6. 8. Castro-G贸mez L, C贸rdova-Ram铆rez S, Duarte-Torres R, Alonso de Ruiz P, Hurtado-L贸pez LM. Cytologic criteria of cystic papillary carcinoma of the thyroid. Acta Cytol. 2003;47:590-4. 9. Kumar S, Singh N, Siddaraju N. Cellular swirls and similar structures on fine needle aspiration cytology as diagnostic clues to papillary thyroid carcinoma: A report of 4 cases. Acta Cytol. 2010;54:939-42. 10. Faser CR, Marley EF, Oertel YC. Papillary tissue fragments as a diagnostic pitfall in fine needle aspirations of thyroid nodules. Diagn. Cytopathol.1997;16:454-9. 11. Gould E, Watzak L, Chamizo W, Albores-Saavedra J. Nuclear grooves in cytologic preparations. A study of the utility of this feature in the diagnosis of papillary carcinoma. Acta Cytol.1989;33:16-20. 12. Ellison E, Lapuerta P, Martin SE. Psammoma bodies in fine needle aspirates of the thyroid: predictive value for papillary carcinoma. Cancer. 1988; 84:169-75.

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Conclusion

The presence of papillary clusters along with characteristic nuclear features on FNAC is well known to indicate a diagnosis of PTC. However, we noted in our study that these findings can be easily missed on cytology smears, especially when the lesions are small (less than 1 cm), and the diagnosis is not apparent clinically. We conclude that presence of papillary clusters on FNAC in a young female patient should alert the pathologist to a possibility of PTC, and trigger a diligent search for nuclear features. Such a practice will help in early detection of PTC even before they become clinically apparent, thus leading to adequate management of the patients and improved prognosis.

Acknowledgements nil

Funding none

Competing Interests none declared

Reference

1. Dean DS, Gharib H. Epidemiology of thyroid nodules. Best Pract Res Clin Endocrinol Metab.2008;22: 901-11.

Original Article Experience of The External Quality Assessment Scheme in Haematology at C.U. Shah Medical College, Surendranagar Killol N. Desai1*, Parth H. Vithalani2, Ashok S. Agnihotri2, Kimmyben Patel3 Department of Pathology, GMERS Medical Collage, Junagadh, India Department of Pathology, C.U. Shah Medical college, Surendranagar, India 3 S.B.K.S Medical Collage and Research Center, Pipariya, Waghodia, India 1

Keywords: External Quality Assessment Scheme; Quality Control; Quality Assurance, Haematology.

ABSTRACT Background: The attainment of quality services in a laboratory requires a comprehensive quality assurance program which includes both internal and external quality control material. External quality assessment scheme programs are accepted around the world as an invaluable tool by laboratories to assess the performance of their testing systems. Results are objectively compared to other laboratories, using the same methodologies for every parameter. Aims: The goal of this study was to review EQAS result from time to time in an effort to improve the performance of the laboratory. Methods: Observational study done at C.U. Shah medical college and hospital, Surendranagar from January 2007 to August 2014. In the current study, our EQAS test results have been evaluated for the past 7 years and 8 months from 2007 to 2014.One EDTA whole blood sample for blood cell counts and 2 slides for peripheral smear examination and reticulocyte count are received quarterly in a year from AIIMS. The test results of all Indices of blood samples and peripheral smears for cell morphology and reticulocyte count were analyzed and documented. Results: Satisfactory results were obtained in all the cycles except thrice. Discrepancy was observed in RBC, MCH and platelet counts. Root cause analysis was performed and necessary action was taken. Conclusion: This participation in EQAS over the last 7 years and 8 months has helped us significantly to improve our laboratory services in terms of performance evaluation, Patient care and overall quality of laboratory practices.

*Corresponding author: Dr. Killol N. Desai, Plot No: 592/1, Sector-3/c, Gandhinagar-382006, Gujarat, India. Phone: +91-9428050253 E-mail: drkilloldesai@gmail.com

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N. Desai et al.

Introduction

External quality assessment (EQA) and proficiency testing (PT) are valuable tools in the quality improvement process. They provide objective evidence of laboratory competence for customers, accrediting bodies and regulatory agencies. It is also important to consider that every EQA/PT scheme has some limitations, and it is not appropriate to use EQA/PT as the sole means for evaluating laboratory performance [1,2]. Therefore, there is a need to underline that internal quality control (IQC). EQA/PT and other tools have to be implemented to monitor and improve the quality in laboratory diagnostics. Programs like this, offer valuable benefits to the participating laboratory, in terms of performance evaluation, improvement in patient care, and the overall quality of laboratory practices[3,4]. The organizing laboratory, that conducts such an EQAS periodically, assesses the registered practicing laboratory. Such a registration is not mandatory, but is desirable. To review and assess the quality of laboratory practices, our hematology laboratory services were registered in 2007 under the ISHTM-AIIMS External quality assurance programme (EQAP), hematology department, AIIMS New Delhi. Since 2007, we are participating, and have been receiving samples four times in a year. Here we share our experience of 7 years and 8 months.

Materials and Methods

Blood samples: EQAS blood samples from All India Institute of Medical Sciences (AIIMS), New Delhi were received and processed at Central Diagnostic Laboratories, C.U. Shah Medical College and Hospital, Surendranagar, Gujarat. For each year, every three months, samples were received at our centre for specific tests recommended by the organizing laboratory. All the samples were handled as a part of routine work samples, and recommended tests were performed by the concerned laboratory technician on duty. The tests were performed on the same day of receipt of the samples, and results mailed to the organizing laboratory within 20 days. Test performed on blood samples: During each cycle, one whole blood EDTA sample for blood cell counts and 2 slides- one for peripheral smear examination, stained by Giemsa stain and one for reticulocyte count, stained by methylene blue were received. We also received a brief clinical summary along with the peripheral smear slide. In our laboratory, the blood samples were run on a Beckman Coulter LH 750 automated cell counter four times, and the highest and the lowest results were selected. All the prints of the histogram were preserved. The stained slides were examined for red blood cell morphology and reticulocyte count respectively. www.pacificejournals.com/apalm

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Result

The following test parameters were tested and documentedRBC count, Haemoglobin, HCT, MCV, MCH, MCHC, Platelet. The peripheral smear slide was examined for the red blood cell morphology and Retic slide for reticulocyte count. All the findings were documented. Table 1: Last eight year EQAS performance Year 2007 2008 2009 2010 2011 2012 2013 2014

Results Unsatisfactory in one cycle Satisfactory Unsatisfactory in one cycle Unsatisfactory in one cycle Satisfactory Satisfactory Satisfactory Satisfactory

Thrice discordant results were obtained in the RBC, MCH and Platelet counts. All three times, root cause analysis was done to find the cause of discordance and random error was found to be the issue. In these instances, the EQAS results were labelled â&#x20AC;&#x2DC;Unsatisfactoryâ&#x20AC;&#x2122;. The samples were then sent to two NABL accredited laboratories for inter-laboratory comparison and results were found to be satisfactory.

Discussion

The EQAS program is a valuable management tool devised to improve the efficiency and service of a laboratory, in particular, and a hospital in general[5].The program provides an opportunity to the participating organizations to compare activities, and modify their own practices, based on what they learn[6,7]. In a clinical laboratory service, EQAS evaluates the performance of procedures, equipment, materials and personnel and suggests areas of improvement. As a participant of EQAS, we performed all the prescribed tests by strictly following the departmental SOPs and manufacturerâ&#x20AC;&#x2122;s instruction, considering each lot as routine working samples. The peripheral smear findings and the reticulocyte count results were satisfactory in all the instances. Discordance arose thrice in the RBC, MCH and Platelets respectively and root cause analysis was performed.

Conclusion

An EQAS program plays an important role in improving the efficiency of a laboratory service, and thereby optimizes the overall quality of a health care system. In the last eight years, we could significantly improve our laboratory services in terms of performance evaluation, patient care by giving accurate result of investigation, follow up eISSN: 2349-6983; pISSN: 2394-6466

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Experience of The EQAS

and overall quality of laboratory practices. We believe that global participation in such an EQAS program will definitely improve the quality of a hospital service, because no health care facility can be totally self-sufficient, and there is always a scope for improvement and development in a system.

Funding Non

Competing Interests None declared

Reference

1. Clinical and Laboratory Standards Institute (CLSI). Using proficiency testing to improve the clinical laboratory; Approved guideline-Second Edition. CLSI document GP27-A2. Clinical and Laboratory standards Institute, Pennsylvania 19087-1898 USA, 2007. 2. Hoeltge GA, Duckworth JK. Review of proficiency testing performance of laboratory accredited by the college of american pathologists. Arch path lab Med 1987;111:1011-4. 3. Miller WG. The role of proficiency testing in achieving standardisation and harmonisation between laboratories. Clin Biochem 2008;42:232-8.

4. Miller WG. Specimen materials, target values and commutability for external quality assessment (proficiency testing) schemes. Clin chim Acta 2004;327:25-37. 5. Sciacovelli I, Zardo I, Secchiero S, Plebani M. Quality specifications in EQA schemes; from theory to practice. Clin Chim Acta 2004;346:87-97. 6. Sciacovelli L, Secchiero , Zardo I, Zaninotto M, Plbani M, External quality assessment : an effective oil for clinical governance in laboratory medicine. Clin Chem Lab Med 2006;44:740-9. 7. Thienpont LM, Stocckl D, Friedecky B, Kratochvilla J, Budina M. Trueness verification in european external quality assessment schemes: time to care about the quality of the samples. J clin Lab Invest 2003;63:195202. 8. Uldall A. Origin of EQA programmes and multidisciplinary cooperation between EQA programme organisers within laboratory medicine. EQA News 1997;8:1-27. 9. Westgard JO. Internal quality control, planning and implementation strategies. Ann Clin Biochem 2003;40:593-611.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Case Report A Rare Renal Neoplasm with Dimorphic Histology and Mucinous Stroma Sunitha Ramachandra*, Lakshmi Rao, Masoud Al Kindi Department of pathology, Armed Forces Hospital, Muscat, Sultanate of Oman Keywords: Kidney tumour, MTSCC, Renal carcinoma

ABSTRACT Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare and recently described histologic variant of renal cell carcinoma (RCC). Usually considered as a tumor of low malignant potential it is important to appreciate the characteristic histologic features to arrive at the correct diagnosis. We report a case of MTSCC in a female aged 54years who presented with a long standing vague loin pain. Diagnostic work-up showed a renal mass suggestive of RCC. With the preoperative diagnosis of RCC followed by nephrectomy showed a completely different histology of a rare tumour with tubules, spindle cells and mucinous stroma consistent with MTSCC, a low grade tumour with good prognosis.

*Corresponding author: Dr. Sunitha Ramachandra, PO Box 726, Seeb PC 111, Muscat, Oman. Phone: +91- 96824331357 E-mail: sunithamanjunath98@yahoo.co.in

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Ramachandra et al.

Introduction

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare subtype of renal cell carcinoma (RCC), a new entity incorporated in the 2004 WHO classification previously described as unclassified RCC.[1] It is believed to be a low grade myxoid tumour with spindle cell change and distal nephron differentiation.[2] Considered to behave in a benign fashion with a good patient prognosis, limited nephrectomy is emphasized.[3] Hence the need to be aware of this unusual neoplasm thus differentiating it from its close mimics of a higher grade RCC.

Case report

A 54-year-old female patient had been suffering with chronic left loin pain since a few years. She had no other complaints and insignificant past history. She presented at our hospital (a tertiary referral centre) with detailed reports of her investigations done elsewhere. CT abdomen revealed a massive heterogenous left renal mass arising from the mid and upper pole with internal echoes compressing the renal parenchyma and involving the renal pelvis. Renal vessels were not involved. This large mass displaced the jejunal loops, descending colon and pancreatic tail. Right kidney, suprarenals, ureters and urinary bladder appeared normal. A radiological diagnosis of a malignant neoplasm with a differential of RCC or TCC (transitional cell carcinoma) was suggested. With the above findings and the possibility of a malignant tumour she was treated with left nephrectomy.

C-143 were noted. Renal pelvis and ureter were free of tumour. IHC (immunohistochemistry) showed epithelial and spindle cells positive for CK 7, Cam 5.2, CK 18, Pan CK and negative for CD 10 and CD 15 (Figure 4). EMA was positive in epithelial cells only. Vimentin showed weak positivity in spindle cells and negative in epithelial cells. The above microscopic and IHC findings were consistent with MTSCC.

Discussion

MTSCC is a rare renal malignant neoplasm recently included in the WHO classification previously called as unclassified renal carcinoma.[1] This is a low grade polymorphic neoplasm composed of bland tubules set in a mucinous stroma with spindle cell areas.[4] The tumour shows a female predominance with 1:4 male-female ratio and wide age distribution ranging between 17 to 82 years with a mean age of 52 years.[1,3] One hundred cases of MTSCC have been reported till-date.[3] Most of them are incidentally diagnosed while being investigated for some other indications.[1] They are usually asymptomatic. A few large tumours present with flank pain, hematuria and anaemia.[1,3] Due to its massive size, our case presented with loin pain and routine blood tests showed low haemoglobin 9.5 gm/dL and reduced red blood cell count (3.6 x 1012/L). Alternately called as low grade collecting duct carcinoma, low grade tubular-mucinous renal neoplasm and low grade myxoid renal epithelial neoplasm with distal nephron differentiation.[5] Majority of these tumours are

Grossly the specimen weighed 1905 grams consisting of a large lobulated soft mass difficult to orient in its anatomic position with a ureteric stump. Cut surface showed a necrotic, solid pale to golden brown tumour replacing the upper and the middle renal parenchyma measuring 16.5 x 14.5 x 12.5 cm involving the renal calices but not the renal pelvis or ureter (Figure 1). The lower pole showed unremarkable renal parenchyma. Suprarenal gland and Gerotaâ&#x20AC;&#x2122;s fascia were not seen. Microscopy showed a well differentiated dimorphic (epithelial and spindle cells) tumour with a pseudocapsule (Figure 2). First component is composed of tightly packed small elongated parallel tubules lined by cells with round nucleus, fine granular chromatin, prominent nucleoli, mild anisonucleosis, eosinophilic cytoplasm and occasional mitoses (Figure 3). Second component is composed of whorls and fascicles of spindle shaped cells with similar morphology. Stroma showed a focus of foam cells, eosinophils, lymphocytes, perivascular plasma cells and thick walled blood vessels. Cystic degeneration with haemorrhage and cholesterol clefts

Fig. 1: Gross picture â&#x20AC;&#x201C; golden brown tumour with necrosis and normal compressed kidney.

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Renal Neoplasm with Dimorphic Histology

Fig. 2: Dimorphic tumour with tubules and spindle cells with myxoid stroma (H&E, 10x)

Fig. 3: Tubules lined by cuboidal cells (H&E, 40 x)

Fig. 4: IHC CK 7 and CK 18 positive epithelial and spindle cells.

incidentally picked up on routine physical examination or during imaging studies. CT imaging shows MTSCC and RCC with slightly different ranges of attenuation values on unenhanced scan. MRI shows similar signal intensity of both the tumour and renal parenchyma on T1-weighted imaging and heterogenous on T2-weighted imaging. FNAB (fine needle aspiration biopsy) may be diagnostic of MTSCC. In such cases the preoperative diagnosis aids in the case management with limited nephrectomy. Our case showed a heterogeneously enhancing lesion with internal hypodense areas and involvement of the renal pelvis(in contrast to the post nephrectomy where renal pelvis was free of tumour). The huge size of the tumour and radiologically involvement of the renal pelvis

probably prompted for a total nephrectomy. The tumour is well circumscribed ranging from 1.0 cm to 18.0 cm in diameter and is usually confined to the kidney.[1,5] Our case measured 16.5 cm in its greatest dimension. Cut surface shows a grey to pale yellow areas often involving the renal medulla.[5] Histology shows a low grade tumour composed of elongated tubules or cordlike growth pattern with branching and freely anastomosing, lined by cuboidal cells with eosinophilic focally vacuolated cytoplasm separated by pale mucinous stroma and spindle cell areas.[2,5] Fine et al classified them as classic with abundant extracellular mucin stroma and mucin poor with little or no extracellular mucin.[3] Tumours may show focal papillary growth pattern, necrosis, ectopic bone and neuroendocrine differentiation

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Ramachandra et al. including carcinoid tumour and small cell neuroendocrine carcinoma.[2,5] Differential diagnosis includes collecting duct carcinoma, papillary RCC solid variant, sarcomatoid RCC, metanephric adenoma and carcinoid. All the above conditions lack a mucinous stroma. Both RCCs show classical areas of RCC whereas collecting duct carcinoma shows highly atypical tubules with desmoplastic stroma and metanephric adenomas are CK 7 negative unlike MTSCC which are CK 7 positive.[4] IHC shows CK 7, CK 19, CK 18, EMA, vimentin and P504S(AMACR) positivity.[5] Histogenesis of the tumour remains debatable and is uncertain. [6] AMACR, CK 7, EMA and vimentin positivity favour a distal tubule origin but AMACR is seen in proximal convoluted tubules as well.[6] Low cuboidal cells, elongated tubular glands and myxoid stroma are reminiscent of loop of Henle.[7] Molecular studies show multiple chromosome losses(1,4,6,8,9,13,14,15 and 22)[2,3] Two cases with neuroendocrine differentiation have been reported.[7] Described as a low grade tumour, histologically is consistent with low grade unless sarcomatoid pattern is noted.[4] Staging is same as RCC. The tumour is always benign and extremely rare to present as metastasis to lymph nodes and other organs. As a subtype of RCC careful follow-up is recommended.

Conclusion

MTSCC is a low grade tumour, a new entity recently included in the WHO classification with good prognosis. One needs to be aware of its classical histological picture and limited nephrectomy can be considered if preoperative diagnosis is available. A massive renal mass excised with a diagnosis of RCC/TCC surprisingly showed a lesion with a new histomorphology and good prognosis favourable to the patient.

Declarations Funds: None

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Competing interests None declared

Referances

1. Lima MS, Barrous-Silva GE, Pereira RA, Ravinal RC, Junior ST, Costa RS and Mugila VF. The imaging and pathological features of a mucinous tubular and spindle cell carcinoma of the kidney: a case report. World J of Surgical Oncology. 2013;11:34 2. Paner GP, Srigley JR, Radhakrishnan A, Cohen C, Skinnider BF, Tickoo SK et al. Immunohistochemical analysis of mucinous tubular and spindle cell carcinoma and papillary renal cell carcinoma of the kidney: significant immunophenotypic overlap warrants diagnostic caution. Am J Surg Pathology. 2006;30:13-9 3. Sun N, Fu Y, Wang Y, Tian T, An W, Yuan T. Mucinous tubular and spindle cell carcinoma of the kidney: A case report and review of the literature. Oncology lettersspandidos publications. DOI: 10.3892/ol.2014.1783 4. Higgins JP and Rouse RP. Mucinous tubular and spindle cell carcinoma of the kidney. Stanford school of medicine. Jan 2011 http://surgpathcriteria.stanford. edu/kidney/mucinous-tubular-spindle-cell-carcinoma/ 5. Williamson S. Mucinous and spindle cell carcinoma. July 2012. www.pathologyoutlines.com/topic/ kidneytumormalignantmucinoustubular.html 6. Wu X, Chen Y, Sha J, Zhao L, Huang J, Bo J et al. Renal mucinous tubular and spindle cell carcinoma: a report of 8 cases and review of the literature. Diagnostic pathology. 2013;8:206 7. Jung SJ, Yoon HK, Chung J, Ayala GA, Ro JY. Mucinous tubular and spindle cell carcinoma of the kidney with neuroendocrine differentiation. Am J of clinical pathology. 2006;125:99-104

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Case Report Solitary Fibrous Tumour of the Prostate with Ossification: A rare Case Report Neeraj Dhameja1, Deepa Santosh1, Vikash1, U.S Dwivedi2 Department of pathology, Institute of Medical sciences, BHU, Varanasi, India Department of Urology, Institute of Medical sciences, BHU, Varanasi, India Keywords: Solitary Fibrous Tumor, Prostate, Fibroblastic, Prognosis

ABSTRACT Solitary fibrous tumour (SFT) is a tumour of fibroblastic origin with characteristic histopathological and immunohistochemical features. Initially described in the pleura, it has now been described in various extrapleural locations including soft tissues and different organs. It is very rare in the prostate with only approximately 30 cases have been described in the literature mostly as case reports. It presents as slowly growing painless mass in the prostate with associated urinary symptoms. Radiologically and clinically it mimics prostatic adenocarcinoma. We report a case of 62 year male patient presenting with obstructive urinary symptoms. Computed tomography imaging suggested a prostatic origin. A trucut biopsy and immunohistochemistry was done which showed features of solitary fibrous tumour which was confirmed on subsequent radical cystoprostatectomy. We review the literature and discuss the challenging issues of misdiagnosis and prognosis.

*Corresponding author: Dr Neeraj Dhameja (Assistant Profesor), Department of pathology, IMS, BHU, Varanasi-221005, India Phone: +91- 7704094687 E-mail: vikas25187@gmail.com

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Dhameja et al.

Introduction

Solitary fibrous tumour (SFT) is a tumour of fibroblastic origin with characteristic histopathological and immunohistochemical (IHC) features. Initially described in the pleura, it has now been described in various extrapleural locations including soft tissues and different organs.[1] It is very rare in the prostate with only approximately 30 cases have been described in the literature mostly as case reports. [2-4] It presents as slowly growing painless mass in the prostate with associated urinary symptoms. Here we describe prostatic SFT in a 62 year male patient presenting with urinary symptoms.

Case Report(S)

A 62 year old male patient presented with dysuria, urinary urgency and increased frequency. CT scan was done which showed a mass in the prostate measuring 18x12x6cm (Fig.1a). Serum PSA levels were within normal limits. Needle biopsy was done which showed fascicles of spindle cells with scattered fibrous bands (Fig.1b). Immunohistochemical stains were done for CD34 and smooth muscle actin (SMA). The tumour cells were strongly and diffusely CD34 positive (Fig.1c) and negative for SMA. Based on these findings a diagnosis of solitary fibrous tumour (SFT) was suggested and excision was done. Because of large size of the tumour, radical cystoprostatectomy was done. On gross examination, the prostate measured 18x12x6cm and was totally replaced by a variegated tumour with solid grey white, cystic,

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C-147 myxoid and bony hard areas. The urinary bladder was grossly unremarkable (Fig.2). Microscopic examination showed a tumour with hypo and hypercellular areas with admixed collagen and many round to ectatic blood vessels giving a hemangiopericytomatous pattern. The tumour was composed of oval to spindle cells arranged haphazardly without any definite pattern (Fig.3a). Foci of ossification were seen (Fig.3b). The hypercellular areas showed necrosis with increased mitotic activity (7/10HPF) focally (Fig.3c and 3d). A panel of IHC stains was performed comprising of CD34, CD99, SMA, CD117 and progesterone receptor (PR). The tumour cells were diffusely and strongly positive for CD34 (Fig.4), weak and focally positive for CD99 and negative for SMA, desmin, CD117 and PR. Based on these characteristic histopathological and IHC findings, a diagnosis of solitary fibrous tumour was made and since the tumour showed increased mitotic activity with necrosis, it was reported as malignant solitary fibrous tumour. Presently the patient is on follow up and symptom free.

Discussion

SFT is a tumour of fibroblastic origin. It was first described in the pleura but now it has been described in various extrapleural locations like soft tissues, subcutaneous tissue, orbit, spinal cord, mediastinum, lungs, thyroid, liver, urinary bladder, testes and prostate.[1] Chan JKC described SFT in pleural and different extrapleural sites (approximately 22 different sites) either related to serosal cavities or unrelated.[5] SFT arising in a prostate is rare and there are few case reports and occasional case series.

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Fig 1 (A) CT scan showing mass in the prostate. (B)Trucut biopsy showing spindle cells with collagen (H&E, 10x). (C) Trucut biopsy showing strong and diffuse CD34 positivity (CD34, 10x).

Fig. 2: Gross specimen showing prostatic tumour and urinary bladder

Fig 3. (A)Low power view showing collagen, haphazardly arranged cells and blood vessels (H&E, 4x). (B) Ossification in hypocellular areas (H&E, 4x). (C) Hypercellular areas with necrosis (H&E, 10x). (D) High power view showing hypercellular areas with increased mitosis (arrows), (H&E, 40x)

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Fig. 4: Tumour cells showing strong and diffuse CD34 positivity in resected tumour (CD34, 20x)

In a series of solitary fibrous tumours of 5 and 12 cases respectively, Westra et al[6] and Mentzel et al[7] described one case each of SFT arising in the prostate. Two cases of prostatic SFT were described by Pins et al[8], one showing benign appearance and the other showing cytological atypia with increased mitotic activity. The largest series comprises of 13 cases by Herawi et al[9] and in this series, 10 cases were arising in the prostate, 2 cases arising between prostate and rectum with extension into the prostate and one case was pelvic mass without prostatic infiltration. About 50% of the cases in this series were malignant. Solitary fibrous tumour in prostate presents as slowly growing mass with associated urinary symptoms like dysuria, urgency and increased frequency. It arises in adult age group with a age range of 46-75 years[9]. Serum prostate specific antigen (PSA) levels are within normal limits. Some patients may present with hypoglycemia as SFT has been shown to produce insulin like growth factors. [1] Grossly, SFT presents as well circumscribed mass with grey white cut surface. Microscopically, it shows oval to spindle cells arranged in a patternless manner with admixed collagen and hemangiopericytomatous blood vessels. The cells are benign in appearance and mitotic activity is low. Ossification can be seen rarely in the hypocellular areas as in the present case.[5] Some SFTs may show hypercellularity with cytological atypia, necrosis and increased mitotic activity (>4/10hpf) and are considered as malignant SFT.[1,5,8] But these criteria are not absolute as there is no correlation between these features and behaviour of the tumour. The most important prognostic factor is complete surgical resection with clear margins. [5] Immunohistochemically, SFT shows strong and diffuse positivity for CD34 and variable positivity for SMA, CD99 but negativity for keratins, PR and CD117. www.pacificejournals.com/apalm

C-149 The differentiation diagnosis includes long list of spindle cell tumours which includes tumours of specialized prostatic stroma and mesenchymal tumours.[8,10] The tumours of specialized prostatic stroma include stromal nodules, stromal tumour of uncertain malignant potential (STUMP) and stromal sarcomas. Stromal nodules are associated with benign prostatic hyperplasia and show benign spindle cells with admixed many small round blood vessels and chronic inflamamtory cells. STUMPs are tumours of prostatic stroma and may show variable patterns like hypercellular stroma with or without atypical cells, phylloides like pattern and myxoid stroma with benign stromal cells.[8,10] Mitosis and necrosis are not seen. Benign prostatic glands are present within the stroma in STUMP but absent in SFT. STUMPs are positive for CD34 and progesterone receptor unlike SFT which are negative for PR. Stromal sarcomas are hypercellular and show cytologically atypical spindle cells with mitosis and necrosis. Sometimes carcinomas of the prostate may show predominantly spindle cell morphology and are called as sarcomatoid carcinomas, however, foci of conventional adenocarinoma or a previous history of prostatic adenocarcinoma is present. The spindle cell tumours of mesenchymal origin include leiomyoma, leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, synovial sarcoma, inflammatory myofibroblastic tumour, hemangiopericytoma and sometimes gastrointestinal stromal tumour. These tumours can be differentitiated from SFT by characteristic histopathological and immunohistochemical features. Leiomyoma and leiomyosarcoma show smooth muscle morphology with benign spindle cells in leiomyoma and atypia and mitosis in leiomyosarcoma and positivity for vimentin, smooth muscle actin and desmin on IHC stain. Rhabdomyosarcoma is seen in younger age group and shows embryonal morphology with desmin and myogenin positivity. Fibrosarcoma and synovial sarcoma morphologically show herringbone and biphasic patterns respectively. Inflammatory myofibroblastic tumours are usually small in comparison of other prostatic spindle cell tumours and show spindle cells in cell culture pattern with admixed inflammatory cells.[10] Sometimes it is very difficult to differentitiate SFT and hemangiopericytoma because the morphological and immunohistochemical features overlap, however, the admixture of collagen and variable cellularity is not seen in hemangiopericytoma. Gastrointestinal stromal tumour (GIST) of the rectum may be confused clinically and radiologically as prostatic mass and histologically shows spindle cells, but can be differentitiated from SFT by positivity for CD117 on IHC in GIST and the characteristic morphology of SFT. The treatment is complete surgical excision with clear resection margins. The behaviour of SFT is variable and eISSN: 2349-6983; pISSN: 2394-6466

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cannot be exactly predicted based on morphological features.

Conclusion

In conclusion, here we have described a case of solitary fibrous tumour in the prostate which is rare with very few cases described. In addition, the present case showed certain unusual morphological features like ossification which has not been described in the prostate. Our case represents the one with complete clinical, radiological and histological data and a long follow up.

Acknowledgements None

Funding None

Competing Interests None declared

Reference

1. Guillou L, Fletcher JA, Fletcher CDM, Mandahl N. Extrapleural solitary fibrous tumour and hemangiopericytoma. In: Fletcher CDM, Unni KK, Mertens F, editors. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press;2002.p8690. 2. Paner GP, Aron M, Hansel DE, Amin MB. Nonepithelial neoplasms of the prostate. Histopathology 2012;60:166-86. 3. Nair B, Nambiar A, Hattangadi SB, Sukumar S, Saifuddin MSC. Solitary fibrous tumour of prostate.

Evaluation and management of a rare tumour. Scandinavian Journal of Urology and Nephrology 2007;41:442-44. 4. Galosi AB, Mazzucchelli R, Scarpelli M, Beltran AL, Cheng L, Muzzonigro G et al. Solitary Fibrous Tumour of the Prostate Identified on Needle Biopsy. European Urology 2009;56:564-67. 5. Chan JKC. Solitary fibrous tumour-everywhere, and a diagnosis in vogue. Histopathology 1997;31:568-76. 6. Westra WH, Grenko RT, Epstein J. Solitary fibrous tumour of the lower urogenital tract: A report of five cases involving the seminal vesicles, urinary bladder and prostate. Human Pathology 2000;31:63-68. 7. Mentzel T, Bainbridge TC, Katenkamp D. Solitary fibrous tumour: clinicopathological, immunohistochemical, and ultrastructural analysis of 12 cases arising in soft tissues, nasal cavity and nasopharynx, urinary bladder and prostate. Virchows Arch 1997;430:445-53. 8. Pins MR, Campbell SC, Laskin WB, Steinbronn K, Dalton DP. Solitary Fibrous Tumour of the Prostate. A Report of 2 Cases and Review of the Literature. Arch Pathol Lab Med 2001;125:274-7. 9. Herawi M, Epstein JI. Solitary Fibrous Tumour on Needle Biopsy and Transuretheral Resection of the Prostate: A clinicopathological study of 13 cases. The American Journal of Surgical Pathology 2007;31:870-76. 10. Hansel DE, Herawi M, Montgomery E, Epstein JI. Spindle cell lesions of the adult prostate. Modern Pathology 2007;20:148-58.

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Case Report Intra-Cystic Papillary Neoplasm of Salivary Gland with Focal Low Grade Malignant Change: A Rare Entity Sohaila Fatima1, Nazima Haider*1, Sabah Nayef Nemri2, Balkur Krishnamoorthi Adiga1 King Khalid University, Guraiger, Abha, Saudi Arabia Aseer Central Hospital, Al Rabwah, Abha, Saudi Arabia 1

Keywords: Salivary Glands, Intraductal Papillary Tumor, Low-Grade Malignant

ABSTRACT Intraductal papilloma is a rare benign salivary gland tumor. It is found in the duct of the minor salivary glands, predominantly in lip and buccal mucosa, but the tumors in the sublingual region are quite rare. Malignant counterpart of intraductal papilloma which is a well defined entity in WHO classification is also extremely rare. This report shows a case of borderline intraductal papillary tumor with low malignant potential. It developed in minor salivary gland of sublingual region. This is a very rare entity, not included in WHO classification of salivary gland tumors and needs to be distinguished from other more common papillary and cystic tumors found in salivary glands.

*Corresponding author: Nazima Haider, King Khalid University, Guraiger, Abha 62529, Saudi Arabia Phone: +91- 966559938735 E-mail: nazima_haider@yahoo.com

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Introduction

Ductal papillomas are a group of relatively rare benign, papillary salivary gland tumors predominantly involving the minor salivary glands. At other end of the spectrum is salivary duct carcinoma which is a well defined entity in WHO classification.[1] Malignant counterpart of intraductal papilloma is extremely rare. Moreover, low grade papillary intraductal salivary gland carcinoma has rarely been described in literature.[2,3] We report a case of borderline, low grade intraductal papillary tumor of sublingual gland in a 50 year old male, an entity not discussed in WHO classification of salivary gland tumors. [1]

Case Report

A 50 year old Saudi male presented in the OPD with the chief complaint of painless mass in the oral cavity on right side for 2 months. There were no other complaints. On examination, a round, well circumscribed submucosal nodule, 3 cm in diameter was palpable in the right sublingual region without fixation. Routine physical examination, biochemical and hematological tests were normal. The excised specimen was sent for histopathological examination. Gross examination showed a grayish white firm mass measuring 2.5x2x2cm. Microscopy showed cystically dilated duct lumen with proliferation of papillary projections lined by two layers of cells-inner mucin secreting epithelial cells and outer myoepithelial cells and having fibrovascular cores (fig. 1). The tumor focally exhibited multilayered lining (fig. 2), with cells displaying moderate pleomorphism, prominent nucleoli

Fig. 1: A part of tumor showing cystically dilated duct lumen with papillary projections lined by two layers of cells and having fibrovascular cores. Inset shows normal salivary gland. (H&E, 5X)

and suspicion of focal stromal invasion (fig. 3, 4) Mitoses were infrequent. Hemorrhage and necrosis was not seen. Smooth muscle actin (SMA) and calponin were positive in most parts of the tumor (fig. 5) indicating presence of myoepithelial cells with focal areas of absence (fig. 6 & 7). The tumor was diagnosed as intra-cystic papillary neoplasm with focal low grade malignant change.

Discussion

Ductal papillomas are a group of relatively rare, benign, papillary salivary gland tumours classified as inverted ductal papilloma, intraductal papilloma, and sialadenoma papilliferum.[1] They represent adenomas with unique papillary features with a common relationship to the excretory salivary duct system, a nonaggressive biologic behaviour, and a predilection for the minor salivary glands. They tend to occur in the middle-aged and elderly and rarely in children.[1] All of the reported sites have been in the minor salivary glandsâ&#x20AC;&#x201D;the most common location is the lower lip followed by the buccal mucosa/mandibular vestibule. Other reported sites have been the palate and the floor of mouth.[4, 5, 6] Intraductal papilloma always occurs in a single cystic space and is characterized by numerous and complex papillary projections. Cytologic atypia and mitotic figures are virtually absent.[1,7] Malignant counterpart of intraductal papilloma has rarely been described in literature.[2,3] Like the papillary neoplasms in other organs such as pancreas, papillary tumors can be classified as benign, borderline and malignant in salivary glands also. However, borderline and malignant intraductal papillary tumors are extremely rare in salivary gland. The cytological and histomorphological criteria remains the

Fig. 2: Papillae showing focal multilayered lining. (H&E, 40X)

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Fig. 3: Tumors cells showing moderate pleomorphism, prominent nucleoli. (H&E, 20X)

Fig. 4: Suspicion of focal stromal invasion depicted by arrows. (H&E, 10X)

Fig. 5: Immunohistochemical study by Smooth Muscle Actin (SMA) showing positivity for myoepithelial cells in papillary projections. (SMA immunostain, 20X)

Fig. 6: Immunohistochemical study by Smooth Muscle Actin (SMA) showing focal negativity (arrow). (SMA immunostain, 10X, Inset 20X)

same in salivary gland intraductal papillary neoplasms such as cytological and nuclear atypia, multilayering, disorganization and invasion.[2,3] Using the similar criteria our case was diagnosed as lowgrade or borderline intra ductal papillary carcinoma. Papillary and cystically dilated duct like structure should be differentiated from other more common papillary and cystic tumors found in salivary glands like salivary duct carcinoma (especially its low-grade variant), the papillary-cystic variant of acinic cell carcinoma, papillary cystadenoma and cystadenocarcinoma, polymorphous low grade adenocarcinoma, and metastatic thyroid papillary carcinoma.[2,3,8] Fig. 7: Immunohistochemical study by Calponin showing focal negativity (arrow). (Calponin immunostain, 20X)

A salivary duct carcinoma is regarded as a high-grade aggressive malignancy of the salivary glands because it has

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invasive growth with early regional and distant metastases with histological similarity to intraductal carcinoma of breast.[9]

Acknowledgements

Histologic variants of a salivary duct carcinoma include a low-grade salivary duct carcinoma, sarcomatoid variant, mucin-rich variant, and invasive micropapillary salivary duct carcinoma.[4,6,10] Low grade variant should especially be differentiated by presence of multifocal intraductal lesions with micropapillary projections rather than papillary projections in single cystically dilated duct as seen in our case.

None

The intraductal micropapillary pattern shows overlapping features with a papillary-cystic acinic cell carcinoma, but tumor cells of an acinic cell carcinoma exhibit basophilic granular or clear cytoplasm rather than eosinophilic cytoplasm, and they show readily identifiable mitotic figures.[5,8] Moreover, the papillarycystic variant of acinic cell carcinoma shows a more delicate fibrovascular core, admixed with a microcystic or follicular structure. [2] The lack of acinar differentiation as seen in our case and negativity for Periodic Acid Schiff (PAS) helps to rule out papillary cystic acinic cell carcinoma.[2] Polymorphous low grade adenocarcinoma may also show papillary structures but forms a mixture of tubular, solid, cribriform, trabecular and targetoid patterns of invasive growth.[1] Metastatic thyroid papillay carcinoma immunoreactivity for thyroglobulin.

shows

Low-grade intraductal carcinomas (LG-IDCs) of salivary gland are rare neoplasms that resemble atypical ductal hyperplasia or LG-IDCs of the breast.[8] Papillary cystadenoma and cystadenocarcinoma are characterised by a neoplastic papillary proliferation of salivary gland duct epithelium in the form of multiple epithelium-lined, multilocular cystic structures.[1,2,8] Therefore, our case is a rare malignant counterpart of intraductal papilloma of sublingual salivary gland showing low grade features. It is different from its closest differentials, low grade salivary duct carcinoma and papillary cystadenocarcinoma.

Conclusion

We want to highlight that although rare, borderline or low grade and malignant intraductal papillary tumors should be considered in the classification of salivary gland tumors.

Nil

Funding Competing Interests None declared

Reference

1. Barnes L, Eveson JW, Reichart PA, Sidransky D, Eds. WHO classification of head and neck tumors. IARC, Lyon: France;2005. 2. Nagao T, Sugano I, Matsuzaki O, Hara H, Kondo Y, Nagao K. Intraductal Papillary Tumors of the Major Salivary Glands: Case Reports of Benign and Malignant Variants. Arch Pathol Lab Med.2000; 124:291-5. 3. Caramanico L, Marcone P, Rovere P, Rocca CD. Intraductal Papillary Carcinoma of the Parotid Gland with Low Malignancy. Pathology- research and practice. 1999; 195: 253–56. 4. Jamal AM, Sun ZJ, Chen XM, Zhao YF. Salivary duct carcinoma of the parotid gland: case report and review of the lîterature. J Oral Maxillofac Surg. 2008; 66: 1708-13. 5. Delgado R, Vuitch F. Salivary duct carcinoma. Cancer.1993;72:1503-12. 6. Nagao T, Gafey TA. Invasive rnicropapillary salivary duct carcinoma. A distinct histologic variant with biologic significance. Am J Surg Pathol. 2004; 28: 319-26. 7. de Sousa SO, Sesso A, de Araújo NS, et al. Inverted ductal papiloma of minor salivary gland origin: morphological aspects and cytokeratin expression. Eur Arch Otorhinolaryngol. 1995; 252: 370–73. 8. Delgado R, Klimastra D. Low grade salivary duct carcinoma. A distinctive variant with a low grade histology and a predominant intraductal growth pattem. Cancer. 1996; 78: 958-67. 9. Lin WL, Ho MC, Chao WR. High-grade salivary duct carcinoma of the parotid gland- case report. J Denl Sci. 2008; 3: 222-27. 10. Nagao T, Watanabe A. Sarcomatoid variant of salivary duct carcinoma. Clinicopathologic and immunoistochemical study of eight cases with review of the literature. Am J Clin Pathol. 2014; 122: 222-31.

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Case Report Cystic Partially Differentiated Nephroblastoma : A Rare Case Report Swagata Dowerah*, Mondita Borgohain Department of Pathology, Assam Medical College. Dibrugarh, India Keywords: Cystic Partially Differentiated Nephroblastoma, Kidney

ABSTRACT Cystic partially differentiated nephroblastoma (CPDN) is a rare tumour of infancy which is considered to be a low risk malignant neoplasm. It has a favourable outcome as compared to classical Wilms tumor. A 10 month old male child presented with abdominal lump of two months duration. Ultrasound abdomen revealed a cystic mass involving most of the left kidney. After nephrectomy we received a specimen of kidney measuring 7x6x5 cms. Multiple cystic areas were seen along with areas of hemorrhage and a narrow rim of normal kidney. On microscopy, multiple cysts of varying sizes were seen lined by cuboidal, flattened, eosinophilic and hobnail cells. The septa were relatively thick containing bland spindle cells with few foci of blastemal cells .A diagnosis of cystic partially differentiated nephroblastoma was made. CPDN is a rare variant of Wilms tumor with a favorable prognosis. Histopathologic examination helps to differentiate it from other cystic lesions of the kidney and is of therapeutic importance.

*Corresponding author: Dr. Swagata Dowerah, Department of Pathology, Basic Science Building,Assam Medical College, Dibrugarh- 786002. India Phone: +91- 9954480337 E-mail: swagatadowerah@gmail.com

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Introduction

Cystic partially differentiated nephroblastoma (CPDN) is a rare tumour of infancy which is considered to be a low risk malignant neoplasm. It has a favourable outcome as compared to classical Wilms tumor. Clinicoradiological differential diagnosis include cystic mesoblastic nephroma, cystic renal dysplasia, cystic nephroma (CN) and Wilms tumor with cystic change. Differentiation from these entities is important due to the therapeutic implications and requires histopathological examination.

Case Report

A 10 month old male child presented with abdominal lump of two months duration. There was no history of jaundice, urinary or bowel abnormalities. Serum biochemical tests were normal. Ultrasound abdomen revealed a cystic mass involving most of the left kidney. A nephrectomy was performed and we received a specimen of kidney measuring 7x6x5 cms on gross examination. Multiple cystic areas were seen along with areas of hemorrhage and a narrow rim of normal kidney. The ureter appeared normal. No lymph nodes were received. On microscopic examination, multiple cysts of varying sizes were seen lined by cuboidal, flattened, eosinophilic and hobnail cells (fig 1,2). The septa were relatively thick containing bland spindle cells. Foci of blastemal cells were seen at places in the septa along with abortive tubule formation (Fig 3). Based on the above findings, a diagnosis of cystic partially differentiated nephroblastoma was made.

Fig. 2: Cysts lines by hobnail cells (H & E, 100X)

Discussion

Cystic renal tumors of infancy are uncommon. Together, they form a spectrum with CN at the benign end, CPDN in Fig. 3: Section showing foci of blastemal cells ( H & E, 40X)

the intermediate region and Wilm’s tumor with multicystic areas at the malignant end [1,2,3]. CPDN is a favorable cystic variant of Wilms’ tumor (nephroblastoma) with unique pathological characteristics. It makes up less than 1% of all Wilms’tumor patients [4].There are two peakage distributions; at 4 months to 2 years and at middle age [5].The tumors are more common in boys than girls (Male:Female = 2:1) [4,6]. Clinically, patients usually present with non specific symptoms such as a painless abdominal mass [7]. Progressive enlargement, which may be sudden, is reported [7,8] . Hematuria can be seen and is thought to be due to the extension of the tumor into the pelvicalyceal system. Fig. 1: Numerous cysts lined by hobnail shaped eosinophilic cells; the septa contain cellular fibrous tissue with bland spindle cells (H&E, 40x)

Grossly, tumor is well circumscribed from the remaining kidney by a fibrous pseudocapsule and consists entirely of cysts of variable size. The septa are thin and there are no

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Dowerah et al. expansile nodules to alter the rounded contour of the cysts. The cysts in cystic partially differentiated nephroblastoma are lined with flattened, cuboidal, or hobnail epithelium, or lack lining epithelium [8]. The septa are variably cellular and contain undifferentiated and differentiated mesenchyme, blastema, and nephroblastomatous epithelial elements [8]. Skeletal muscle and myxoid mesenchyme are present in the septa of most tumours. Cartilage and fat are present occasionally .[5,9] In 1977, Joshi and Banerjee [8] distinguished CPDN from CN depending on the presence and absence of blastemal element within the septa, respectively. Other authors propose that CN is a CPDN in which the blastemal elements maturated or that Wilm’s tumor with multicystic areas is simply a precursor for CPDN. [5] The presence of blastemal cells or poorly differentiated stromal or epithelial elements should exclude the diagnosis of Multilocular cyst or CN. On gross examination, Wilm’s tumor with multicystic areas contains expansile nodules. In 1989 Joshi et al.[5,8,9] described diagnostic criteria for CPDN and recommended using the term CN instead of multilocular cyst ( MLC) when the septa contained mature tubular structures. The presence of blastemal cells or poorly differentiated stromal or epithelial elements should exclude the diagnosis of MLC or CN. The diagnostic criteria by Joshi et al.[5,8] for CPDN include: the tumor composed entirely of their septa; discrete well demarcated mass; septa being sole solid component and conform to outlines of cysts without expansile nodules; cysts being lined by flattened, cuboidal or hobnail epithelium; and septa containing blastema and/or embryonal stroma or epithelium element. It is important to distinguish CPDN from the other cystic renal tumors especially Wilm’s tumor with multicystic areas since the latter should receive adjuvant chemotherapy. On the other hand, CPDN is curable by nephrectomy alone. [10] [11] However, a regular follow up is recommended to document any recurrence.

Conclusion

CPDN is a rare variant of Wilms tumor with a favorable prognosis. Histopathologic examination helps to differentiate it from other cystic lesions of the kidney and is of therapeutic importance.

Acknowledgements none

Funding None

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Competing Interests None declared

Reference 1. Bindhu J,Imtiaz A, Kumar RV, Thejaswini MDRT. Cystic variant of favorable-histology Wilms’ tumor presenting with osteolytic metastasis to the ribs. J Postgrad Med. 2010,56:28-30. 2. Eble JN, Bonsib SM. Extensively cystic renal neoplasms: cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma and cystic hamartoma of renal pelvis. SeminDiagnPatho. 1998;15:2-20. 3. Parikh KS, Shukla NS, Shah MP et al. Cystic partially differentiated nephroblastoma. Indian J Med PediatrOnco. 2004; 24:34-7. 4. Blakely ML, Shamberger RC, Norkool P, et al. Outcome of children with cystic partially differentiated nephroblastomatreated with and without chemotherapy. J Pediatr Surg. 2003; 38: 897-900. 5. Joshi VV, Beckwith JB. Multilocular cyst of the kidney (cystic nephroma) and cystic partially differentiated nephroblastoma. Terminology and criteria for diagnosis. Cancer. 1989; 64: 466-79. 6. Singh S, Gupta R, Khurana N. Cystic partially differentiated nephroblastoma: a rare differentiated variant of Wilm’s tumour. J Postgrad Med. 2006; 52:45-6. 7. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologic-pathologic correlation. RadioGraphics. 1995; 115:659-660. 8. Joshi VV, Banerjee AK, Yadav K, Pathak IC. Cystic partially differentiated nephroblastoma: a clinicopathologic entity in the spectrum of infantile renal neoplasia. Cancer. 1977; 40: 789-795. eISSN: 2349-6983; pISSN: 2394-6466

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Cystic Partially Differentiated Nephroblastoma

9. Joshi VV, Beckwith JB. Pathologic delineation of the papillonodular type of cystic partially differentiated nephroblastoma. A review of 11 cases. Cancer. 1990; 66:1568-77. 10. Rajangam K, Narasimhan KL, Trehan A, et al. Partial nephrectomy in cystic partially

differentiated nephroblastoma. J Pediatr Surg. 2000; 35:897-900. 11. Sonia KP, Shilin NS, Pankaj MS, et al. Cystic partially differentiated nephroblastoma. Indian J Med Pediatr Oncol. 2004; 25:34-7.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Case Report Eccrine Angiokeratomatous Hamartoma: Report of A Case with Brief Review Vijayashree Raghavan, Devi Subbarayan, Ramesh K. Rao* Department of Pathology, Chettinad Hospital and Research Institute, Tamilnadu, India Keywords: Eccrine Angiokeratomatous Hamartoma, Eccrine Angiomatous Hamartoma, Solitary Angiokeratoma, CD34, SMAa, Cytokeratin

ABSTRACT Eccrine angiokeratomatous hamartoma is a newly recognised vascular lesion of the skin, with the first case being reported only in 2006. It has a distinctive histology with features derived from two well established vascular lesions affecting the skin: solitary angiokeratoma and eccrine angiomatous hamartoma. A 26 year old woman presented with a long standing painless warty lesion on her right ankle. Histological examination of the excised lesion showed a combination of two vascular lesions: superficial angiokeratoma and a deeper eccrine angiomatous hamartoma. Such a histological picture is consistent with the diagnosis of recently described eccrine angiokeratomatous hamartoma. Our case represents only second such case to be reported. Immunohistochemical analysis with CD34, smooth muscle actin and cytokeratin was done.

*Corresponding author: Dr. K. Ramesh Rao, Professor and HOD, Department of Pathology, Chettinad Hospital and Research Institute, Tamilnadu 603103 INDIA Phone: +91-9884616318 E-mail: rameshkrao@gmail.com

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Eccrine Angiokeratomatous Hamartoma

Introduction

Eccrine angiokeratomatous hamartoma (EAKH) is a newly recognised vascular lesion of the skin, with the first case being reported only in 2006[1]. It has a distinctive histology with features derived from two well established vascular lesions affecting the skin: solitary angiokeratoma (SAK) and eccrine angiomatous hamartoma (EAH). Solitary or multiple angiokeratoma is a very common condition that presents as a single or many papular lesions on the extremities of young adults [2]. EAH is relatively rare lesion that is usually present at birth or manifests early in the childhood as multiple nodules or as a single large plaque on the extremities [3]. Since its recognition, no other case of eccrine angiokeratomatous hamartoma has been documented until now. We are reporting second such case.

Case Report

A 26 year-old woman presented with a painless, fleshcoloured warty lesion over the lateral malleolus of her right ankle. She had no history of trauma or surgery in the recent past and nor did she suffer from any other major illness. The patient first noticed this lesion when she was 4 years old, when it apparently started as a tiny painless nodule. Since then, it had grown very gradually to its present size of 3 cm. On several occasions in the past, it ulcerated following trivial trauma and was accompanied by pain, tenderness and crusting. No one else among her immediate family had similar lesion. The lesion was surgically excised and was submitted for hisopathological examination. The excised specimen consisted of a thick elliptical piece of skin with subcutaneous tissue. It measured 3.5x2x2 cm. The skin was verrucous. On bisection, the cut surface was fairly homogeneous tanwhite, but no clearly demarcated lesion was seen.

Fig. 1: From the superficial part of the lesion showing Angiokeratoma-like area (Haematoxylin and Eosin, 100X)

Entire specimen was processed for hisopathological examination. Besides the routine haematoxylin and eosin (H&E) staining, the sections were also subjected to immunohistochemistry with antibodies against CD 34, cytokeratin, desmin and smooth muscle actin-a (SMA-a) [Novocastra (Leica) Mouse monoclonal antibodies]. On microscopic examination, the epidermis showed verrucous hyperplasia. But the histology was dominated by a poorly delineated vascular lesion with two distinctive morphological areas: The superficial part, which involved papillary and superficial reticular dermis, was composed of thin walled, partly ectatic capillary vessels, which at places were partly enclosed within an epidermal collarette. Some of the larger vessels were occluded by fresh intraluminal fibrin thrombi. This portion had the features of solitary angiokeratoma The deeper portion had a distinctive lobular configuration and affected the mid and deep reticular dermis. It was composed of fairly uniform thick walled capillaries intricately admixed with sweat ducts and glands. Some of the lobules contained adipose tissue with myxoid interstitium. Some of the deeper vessels showed intraluminal fibrin thrombi. This part had the histological features of eccrine angiomatous hamartoma. Immunohistochemically, the proliferating blood vessels in both areas were positive for CD 34 (figure 3 A & B) and smooth muscle actin-a (figure 4 C & D). The latter was positive either as a continuous or discontinuous single layer within the walls of vessels in the angiokeratomatous area. In the deeper eccrine angiomatous hamartomatous area, it was thick, multi-layered and more abundant. Desmin was negative in all the proliferating vessels. Cytokeratin delineated the eccrine glands and ducts in the deeper part (figure 5).

Fig. 2: From the deeper part of the lesion showing Eccrine Angiomatous Hamartoma-like area (Hearmatoxylin and Eosing, 100X)

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Fig. 3: Immunohistochemistry for CD34. Blood Vessels in superficial (A) and deeper (B) portions of the lesion are strongly positive

Fig. 5: Immunohistochemistry for cytokeratin. Sweat glands and ducts in eccrine angiomatous hamartoma-like area are positive.

Discussion

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Fig. 4: Immunohistochemistry for SMA-alpha. Blood vessels in superficial (A) and deeper (B) portions of the lesion are strongly positive

varying sizes (figure 1). But the deeper EAH-like areas had fairly uniform small thick walled vessels resembling arterioles (figure 2) unlike what was reported by Kanitakis et al [1]. Immunohistochemically, the vessels in both areas expressed CD 34 and SMA-a but the intensity of staining for SMA-a was more in the EAH-like area (figure 3 & 4). As SMA-a is expressed by not only smooth muscle cells but also by the pre-and post-capillary pericytes and myofibroblasts [4], immunohistochemistry for desmin was done, as it is expressed only by muscle cells and can be used to distinguish between pericytes and smooth muscle cells. All the proliferating vessels in our case were negative for desmin. So, even though the vessels in deeper portion appeared thick walled, they did not have any smooth muscle in their walls and the thickness observed is probably due to proliferation of pre- and post- capillary pericytes. In both areas, some of the blood vessels were occluded by fresh fibrin thrombi, a feature frequently observed in SAK [2].

In the present case, the patient noticed the lesion as a small nodule when she was 4 years old. Its progression was very slow and was punctuated by several episodes of painful ulcerations. At the time of presentation, the lesion measured only about 3 cm and was painless. Histologically, as we have described, it exhibited features of both angiokeratoma and eccrine angiomatous hamartoma. The blood vessels in these two areas were morphologically different. The angiokeratomatous areas had thin, ectatic capillaries of

The differential diagnosis for this would be EAH and SAK. Eccrine angiomatous hamartoma (EAH) is a relatively rare condition that usually manifests at birth or early in childhood as a papule or a nodule or a plaque on distal portions of the extremities [5] [6] [7] [8]. But they have been reported on other sites including head and neck region and trunk [9] [10]. It has also been reported in adults [11]. It commonly presents as a solitary lesion, but occurrence of multiple EAH has been documented [12] [13]. The lesion is usually asymptomatic but may be associated with pain and hyperhidrosis [14]. Histologically the lesion affects mainly mid and deep dermis and is composed of lobules of proliferating capillaries intricately admixed with sweat glands and ducts, fat and myxoid tissue. Immunohistochemically, the sweat glands have been shown to be positive for S 100, EMA and cytokeratin; and the vascular component for CD 31 and CD 34.

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EAKH was recognised as a distinct new entity in 2006 by Kanitakis et al [1] when they discovered that a case clinically diagnosed as EAH had histological features of both SAK and EAH. They proposed the new name as well. In their case too, the clinical presentation was similar to the present case and the lesion was seen on the right malleolus. It was small but tender.

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Eccrine Angiokeratomatous Hamartoma

Solitary or multiple angiokeratoma (SAK) is a common condition that tends to affect the lower extremities of young adults. However, it has been reported from other sites as well including oral cavity [15]. It usually presents as solitary or multiple papular lesions. The histology is characterised by the presence of thin ectatic capillary vessels in papillary dermis with overlying epidermis showing verrucous hyperplasia. The rete ridges at places may form epidermal collarette. Some of the vessels may show fresh thrombi and the vessels are usually positive for CD 31 and CD 34. Since its recognition in 2006, no other case of EAKH has so far been documented. As our case meets the morphological diagnostic criteria of EAKH, it represents only the second such case to be recognised. Paucity of documentation reflects either the rarity of this condition or lack of awareness. Whether it is distinctive entity or a variant of EAH or SAK can only be established if a few more cases are reported.

Conclusion

We are presenting only the second documented case of eccrine angiokeratomatous hamartoma having morphological features of both angiokeratoma and eccrine angiomatous hamartoma. Whether it is a distinctive entity or a variant of those two lesions remains to be established.

Acknowledgements

Management of CHRI for permitting this article to be published

Funding None

Competing Interests None declared

References

1. Kanitakis J, Ly A, Claudy A. Eccrine angiokeratomatous hamartoma: A new variant of eccrine hamartoma with angiokeratoma. J Am Acad Dermatol. 2006; 55: S104-6. 2. Imperial R, Helwig EB. Angiokeratoma. A clinicopathological study. Arch Dermatol. 1967; 95:166-75 3. Hyman AB, Harris H, Brownstein MH. Eccrine angiomatous hamartoma. N Y State J Med. 1968; 68:2803-6.

4. Nehls V, Drenckhahn D. Heterogeneity of Microvascular Pericytes for Smooth Muscle Type Alpha-Actin. J Cell Biol. 1991; 113:147-54. 5. Martinelli P, Tschen J. Eccrine angiomatous hamartoma: a case report and review of the literature. Cutis. 2003; 71:449-55. 6. Pelle M, Pride H, Tyler W. Eccrine angiomatous hamartoma. J Am Acad Dermatol. 2002; 47:429-35. 7. Kar S, Krishnan A, Gangane N. Eccrine Angiomatous Hamartoma: A Rare Skin Lesion with Diverse Histological Features. Indian J Dermatol. 2012; 57:225-7. 8. Smith V, Montesinos E, Revert A, Ramon D, Molina I, Jorda E. Eccrine angiomatous hamartoma: report of three patients. Pediatr Dermatol. 1996; 13:139-42. 9. Yi-Ting Lin, Chien-Ming Chen, Chih-Hsun Yang, Yea-Huey Chuang. Eccrine Angiomatous Hamartoma: A Retrospective Study of 15 Cases. Chang Gung Med J 2012; 35:167-77. 10. Shin J, Jang YH, Kim S, YC K. Eccrine Angiomatous Hamartoma: A Review of Ten Cases. Ann Dermatol. 2013; 25:208-21. 11. Natarajan K, Rai R, Sundararajan V, Venkatchala S. Eccrine angiomatous hamartoma in an adult. Indian J Dermatol Venereol Lepr. 2009; 75:193-4. 12. Ghosh SK, Bandyopadhyay D, Chatterjee G. Multiple Eccrine Angiomatous Hamartoma in a Young Boy: A Case Report. Turk Acad Dermatol 2008; 2 (2). Retrieved fromhttp://www.jtad.org/2008/2/ jtad82201c.pdf 13. Naik V, Arsenovic N, Reed M. Eccrine angiomatous hamartoma: A rare multifocal variant with features suggesting trauma. Dermatology Online Journal. 2009; 15(9). Retrieved from: https://escholarship.org/ uc/item/3fd7h8bs 14. Challa VR, Jona J. Eccrine angiomatous hamartoma: A rare skin lesion with diverse histological features. Dermatologica. 1977; 155:206-9. 15. Kandalgaonkar S1, Tupsakhare S, Patil A, Agrawal G, Gabhane M, Sonune S. Solitary Angiokeratoma of Oral Mucosa: A Rare Presentation. Case Rep Dent. 2013; 2013:812323.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Case Report Primary Chondrosarcoma of The Breast: A Rare Case Presentation Anshu Jain, Veena Maheshwari, Shagufta Qadri, Divya Rabindranath*, Nishat Afroz Department of Pathology, J.N. Medical College, A.M.U. Aligarh, India Keywords: Breast, Chondrosarcoma, Cytology, Primary

ABSTRACT Pure sarcomas are very uncommon tumors of the breast. Only ten case reports of primary chondrosarcoma of breast have been published in literature till date. We report this rare case highlighting the cytological features which helped in a preoperative diagnosis of this tumor. A 40 year old lady presented with lump and pain in right breast. She did not have any other significant findings. Fine needle aspiration cytology yielded abundant material, comprising of benign ductal epithelium along with malignant cartilaginous component. Subsequent histopathology and immunohistochemistry confirmed the cytological diagnosis of primary chondrosarcoma of breast. Primary chondrosarcoma of breast, even though a rare entity, should be kept in the differential diagnosis of breast tumours exhibiting chondrosarcomatous areas.

*Corresponding author: Dr. Divya Rabindranath, Department of Pathology, J.N. Medical College, A.M.U., Aligarh-202002. Uttar Pradesh, India. Phone: +91- 7895683197 E-mail: divy30@hotmail.com

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A Rare Case of Primary Breast Chondrosarcoma

Introduction

Primary pure sarcomas of breast represent only 0.5% of breast malignancies.[1] The differential diagnosis includes metaplastic breast carcinoma with chondrosarcomatous differentiation and cystosarcoma phyllodes.[2] It is important that they are recognized as a separate entity from the more common breast carcinomas and the difference in behavior of these two tumors is taken into account when planning therapy.[1]

Case Report(S)

A 40 year old Asian female presented with right breast lump since one year. She had developed pain in the lump since seven days. There was no history of lump or pain anywhere else in the body. There was no family history of breast cancer and patient was multiparous. On examination, there was an 8 x 8 cms hard lump in the right breast. Overlying skin was unremarkable, apart from few prominent veins. Lump was not fixed to underlying chest wall. No axillary or supraclavicular lymphadenopathy was noted. Left breast was normal. Rest of the systemic and general examination was unremarkable. Her routine laboratory investigations were normal. A chest radiograph was also normal with normal ribs. A provisional clinical diagnosis of carcinoma breast was made and patient was advised fine needle aspiration cytology (FNAC). Multiple site sampling from the mass was done. The FNAC smears were highly cellular and showed sheets of benign ductal cells along with loose aggregates and scattered population of moderate to markedly atypical cells against a chondroid background (Figures 1a, 1b). At places typical chondroblastic morphology was also appreciable (Figure 1c). Only occasional stromal fragments and few mitosis were noted. This led to a differential diagnosis of chondrosarcoma of breast. A diligent search for any chondrosarcomatous lesion in bones was made,

and none found. Patient underwent right modified radical mastectomy with axillary lymph node dissection. On grossing, the mastectomy specimen measured 15x9x4 cm with overlying ellipse of skin measuring 11x8 cm. Cut sections showed a tumor 10x7x6 cm in size. It was whitish, friable with foci of cystic change along with chondroid areas (Figure 2a). Tumor distance from all margins was â&#x2030;Ľ1.0 cm. 10 lymph nodes were dissected out, none of which seemed involved grossly. Extensive sampling for histopathological examination was done. Microscopically the tumor was composed entirely of lobules of chondroid material with moderate to markedly atypical chondrocytes (Figures 2b, 2c). These lobules were separated by fibrotic stroma. The tumor showed involvement of adipose tissue of breast. Benign ductal component could be seen at the periphery of tumor. Immunohistochemically, the tumor cells were S 100 positive (Figure 3a); Pan Cytokeratin, Estrogen Receptor, Progesterone Receptor, & Her2 neu negative (Figures 3b, 3c, 3d, 3e). Skin, all margins & the lymphnodes were free from tumor. A final diagnosis of primary chondrosarcoma of breast was made and as it was fully excised, patient was kept under observation with regular followup. She is doing well six months after surgery.

Discussion

Most pathologic lesions in the breast arise from the epithelium. The stroma, however, may also give rise to neoplasms; in general, these are similar in appearance to mesenchymal lesions seen at other sites. Pure sarcomas, without an associated epithelial component, occasionally occur in the breast and should be distinguished from metaplastic carcinoma and malignant phyllodes tumor that has outgrown its epithelial component.[3] Fewer than 150 cases of primary osteosarcoma of breast have been reported in the medical literature. Primary

Fig. 1[a]: Photomicrograph showing branching sheet of benign ductal cells (upper half) and malignant chondroid component (lower half). (H&E, x100); [b] Benign ductal cells in close proximity with atypical malignant cells with a chondroid background. (H&E, x400); [c] Typical chondroblast with surrounding lacunar space and background chondroid material. Adjacent atypical cells can be confused with malignant ductal component, and erroneously diagnosed as ductal carcinoma if attention to chondroblasts is not given. (H&E, x400).

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Fig. 2 [a]: Gross photograph of cut section of tumor showing a whitish and friable appearance; [b] Lobules of chondroid material with atypical chondroblasts. (H&E, x100); [c] Higher power showing atypical nuclear morphology as well as hypercellularity. (H&E, x400).

Fig. 3: Immunohistochemistry of tumor cells showing [a] S100 positivity (x400); while negativity for [b] Pan Cytokeratin (x400), [c] ER (x100), [d] PR (x100), & [e] Her2 neu (x400).

chondrosarcoma is even rarer and publications are limited to isolated case reports or few cases in series encompassing a heterogenous group of mammary sarcomas.[4] The existence and histogenesis of primary mammary matrix producing (MP) sarcomas, namely osteosarcoma and chondrosarcoma, remains controversial. It is still unidentified whether these represent the end stage of transdifferentiation towards osteochondroid lineage with loss of residual epithelial components (carcinoma); or these are tumors arising from totipotent mesenchymal cells of the breast stroma (sarcoma).[4]

background matrix material and typical chondroblastic morphology present focally. Only occasional stromal fragments were seen which is non-favourable for phyllodes tumor, although it is very difficult to distinguish only by cytology. Even on histopathology, it is sometimes very difficult to distinguish a primary chondrosarcoma from metaplastic breast carcinoma and malignant phyllodes tumor, if the chondrosarcomatous component is predominant. In such cases wide sampling is important in order to rule out any foci of in situ or invasive epithelial malignancy. Any such foci would lead to a diagnosis of metaplastic breast carcinoma.[3]

A preoperative clinical and cytological diagnosis in such cases is usually not reached, both due to marked similarity in clinical behaviour to infiltrating ductal carcinoma and low index of suspicion.[2] The cytological findings of primary breast chondrosarcoma have never been elucidated before . In our case, the presence of large amount of benign ductal component indicated a nonepithelial nature of the malignancy and raised the strong suspicion thereby confirming the retrospective diagnosis of primary chondrosarcoma on cytological basis. The malignant chondroid component can be mistaken for ductal carcinoma, however one should pay attention to the

In the occasional case reports, these tumors have usually been found to be large in size, occur in women more than 40 years old, and usually do not invade the overlying skin. [5] Regional lymphadenopathy is expected in 14-29% of these cases, most of which are reactive hyperplasias.[6] Microscopically the tumor shows chondroid areas with cellular atypia and pleomorphism.[2] Differentiation from metaplastic carcinoma is possible by absence of direct transition between carcinomatous and mesenchymal component in the former. Further the sarcoma like

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A Rare Case of Primary Breast Chondrosarcoma

elements in metaplastic carcinoma though acquire vimentin positivity, still retain epithelial markers.[7]

Funding

Differentiation form malignant cystosarcoma phyllodes with predominant chondrosarcomatoid component can be extremely difficult. It has been mentioned that benign ductal elements interspersed- among sarcomatoid areas should be taken as evidence of former. Most mammary tumours with areas of chondroid metaplasia show benign histological appearance.[2] Another important differential diagnosis that should be considered in such cases is primary sarcoma of the chest wall metastasizing to the breast. The only method to rule out this possibility is thorough search for lesions in chest wall preoperatively through imaging; and during surgery.

Competing Interests

Surgery remains the treatment of choice for most sarcomas. The role of chemotherapy and radiotherapy is not yet established because of the limited number of cases reported so far.[1]

Conclusion

In conclusion, a preoperative diagnosis in a case of primary chondrosarcoma of the breast is important as it would surely negate the need for un-necessary preoperative chemotherapy to the patient. This is important because chondrosarcomas are primarily managed surgically, and generally have good prognosis once completely excised as compared to the ductal carcinomas.

Acknowledgements Nil

None

None declared

Reference

1. Errarhay S, Fetohi M, Mahmoud S, Saadi H, Bouchikhi C, Banani A: Primary Chondrosarcoma of the breast: a case presentation and review of the literature. World Journal of Surgical Oncology 2013,11: 208-11. 2. Gupta S, Gupta V, Aggarwal PN, Kant R, Khurana N, Mandal AK: Primary Chondrosarcoma of breast: A case report. Indian J Cancer 2003; 40: 77. 3. Carter D, Schnitt SJ, Millis RR: The Breast. In: Mills SE, editor. Sternbergâ&#x20AC;&#x2122;s Diagnostic Surgical Pathology. Philadelphia: Lippincott Williams & Williks; 2010. p 325-350 4. Rakha EA, Tan PH, Shaaban A, Tse GM, Esteller FC, van Deurzen CHM et al: Do primary mammary osteosarcoma and chondrosarcoma exist? A review of a large multi-institutional series of malignant matrixproducing breast tumors. The Breast 2013; 22: 13-18. 5. Kennedy T, Biggart JD: Sarcoma of the breast. Br J Cancer 1967;21:635-44. 6. Barnes L, Pietruszka M: Sarcoma of the breast A clinico pathologic analysis of ten cases. Cancer 1977;40:1577-85. 7. Vandenhaute B, Validire P, Veilleux C, Voelh P, Zafrani B: Breast carcinoma with chondroid metaplasia. Ann Pathol 1995;15:53-8.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Case Report Cytology: A Diagnostic Modality in Cryptococcal Lymphadenitis Pooja Srivastava, Kusum Gupta, Rashmi Arora, Department of Pathology VMMC and Safdarjung Hospital, New Delhi, India Keywords: Cryptococcus, Cytology, Lymphadenitis, Fungus

ABSTRACT Cryptococcosis is a common, life-threatening, opportunistic, fungal disease in human immunodeficiency virus (HIV) infected individuals. A prompt diagnosis is of utmost importance because disseminated cryptococcal infection is lifethreatening. Different organs can be involved, but involvement of lymph node is a rare feature. We here present a case of cryptococcal lymphadenitis diagnosed on fine needle aspiration cytology.

*Corresponding author: Dr Pooja Srivastava, 270, Hauz Rani Malviya Nagar, New Delhi, India 110017 Phone: +91- 9015354141 E-mail: panacea.2417@gmail.com

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Cytology in Cryptococcal Lymphadenitis

Introduction

Cryptococcosis is a common opportunistic fungal disease seen in human immunodeficiency virus (HIV) infected individuals. It is caused by the encapsulated yeast cryptococcus neoformans.[1] Usually affected sites include respiratory and central nervous system. Lymph node involvement as a presenting feature is not a common manifestation of the disease.[2] Disseminated cryptococcosis is a life-threatening disease [3] hence a prompt and early diagnosis is mandatory. We report here a case of cryptococcal lymphadenitis that was diagnosed by fine-needle aspiration cytology (FNAC) of the involved cervical lymph node.

Case Report

A 25 year old male presented with complaints of fever, cough, generalized weakness and significant weight loss for 2 months duration. Patient was a known case of HIV infection and was on treatment. On examination, he was of thin built and had palpable cervical lymphnode measuring 1x1 cm. His systemic examinations, routine hematological as well as biochemical investigations were almost within normal limits. Based on these findings a presumptive clinical diagnosis of tuberculosis was made and fine needle aspiration from the enlarged lymphnode was performed. Giemsa stained smears demonstrated numerous spherical yeast cells of variable size surrounded by a clear halo [Figure 1] with few lymphocytes and histiocytes in a necrotic background. No epithelioid cells were seen. No acid-fast bacilli could be seen on Ziehl-Neelsen-stained smears. Periodic acid–Schiff (PAS) stain [Figure 2] was

Fig. 1: Smear showing spherical, budding, encapsulated yeasts with thick (clear zone) capsule (Giemsa, 100x).

put which facilitated the identification of organism. Thus, possibility of cryptococcal infection was considered and patient was started on antifungal treatment to which he adequately responded. Patient was further followed up for two months after which he was lost to follow up.

Discussion

Cryptococcosis is a chronic opportunistic infection caused by the encapsulated yeast cryptococcus neoformans. [1] It was first described in the 1890s though its increased prevalence was reported in the early 1980s owing to HIV pandemic.[4] Cryptococcus lymphadenitis is an unusual form of extrapulmonary cryptococcosis and is one of the ‘AIDS defining criteria’ as per Centre for Disease Control and Prevention (CDC) guidelines. [3,5] Various cytological studies of HIV lymphadenopathy have indicated that tuberculosis is the most frequent infection occurring in HIV patients in India.[6] The clinical presentation in the form of lymph node enlargement makes tuberculosis as the first differential diagnosis, especially because of its high prevalence in India. The diagnosis of cryptococcus can be made microscopically by demonstrating yeast cells in lymph node aspirate. The yeast cells are surrounded by a thick halo-like capsule measuring 5-15μm in diameter. Special stains like gomori’s methanamine silver, PAS and mucicarmine can be used to facilitate the identification of organism. [1] Although, culture is important for identification of the pathogen, diagnosis of cryptococcosis can be made on cytologically obtained smears when the mucopolysaccharide capsule is visualised with aid of special stains. [3]

Fig. 2: Periodic acid–Schiff (PAS) stain highlighting the presence of cryptococcus neoformans (PAS, 200x).

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Conclusion

FNAC is a safe, simple and useful technique which can expedite an accurate and rapid diagnosis of cryptococcal lymphadenitis, thereby helping in timely initiation of treatment.

Acknowledgements Nil

Funding None

Competing Interests None declared

Reference

1. Sainath AK, Ahuja M, Shaik I. Cryptococcal Lymphadenitis on Fine Needle Aspiration Cytology. Peopleâ&#x20AC;&#x2122;s Journal of Scientific Research 2012;5:33-5.

C-169 a chance diagnosis by FNAC. Diagn Cytopathol 2013;41:456-8. 3. Suchita S, Sheeladevi CS, Sunila R, Manjunath GV. Fine needle aspiration diagnosis of cryptococcal lymphadenitis; A window of opportunities. Journal of Cytology 2008;25:147-9. 4. Philip KJ, Kaur R, Sangeetha M, Masih K, Singh N, Mani A. Disseminated cryptococcosis presenting with generalized lymphadenopathy. J Cytol 2012;29: 2002. 5. Tahir M, Sharma SK, Sinha S, Das CJ. Immune reconstitution inflammatory syndrome in a patient with cryptococcal lymphadenitis as the first manifestation of acquired immunodeficiency syndrome. J Postgrad Med 2007;53:250-2.

2. Bhuyan P, Pattnaik K, Kar A, Brahma RC, Mahapatra S. Cryptococcal lymphadenitis in HIV:

6. Anvikar AR, Gosavi AV, Kulkarni MP, Lanjewar DN. Cytodiagnosis of coexistent cryptococcal and mycobacterial lymphadenitis in a case of AIDS. J Cytol 2011; 28:25â&#x20AC;&#x201C;7.

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Case Report Pleural Effusion Obscuring Pulmonary Echinococcosis: Diagnosis by Cytology Anshu Jain, Veena Maheshwari, Divya Rabindranath*, Kashmi Sharma Department of Pathology, J.N.Medical College, A.M.U., Aligarh, India Keywords: Echinococcus, Pleural Effusion, Hydatid Cyst, Cytology

ABSTRACT Echinococcosis or hydatid disease, caused by larvae of the tapeworm Echinococcus, is frequently seen in an endemic country like India. A single organ is involved in 85-90% cases, pulmonary involvement occurs in 10 - 30% of cases, being second only to the hepatic involvement. Besides anaphylactic reactions a pulmonary hydatid cyst may be complicated by rupture into neighbouring structures like bronchi and pleural cavity with or without infection. We report an unusual case of ruptured hydatid disease in pleural cavity rarely seen in adults diagnosed by fine needle aspiration cytology. A 45 year old male came to our emergency department in shock with symptoms of shortness of breath and altered mental status from the previous day. Radiograph showed massive left sided pleural effusion and therapeutic aspiration was done. Pleural fluid cytology showed few hydatid scolices. Serum echinococcal antigen was also tested to be positive. CT scan revealed pleural effusion with multiple air pockets and pleural thickening on left side, and loculated cyst with septae on right side, thereby confirming the diagnosis as active pulmonary hydatid cyst with rupture into the pleural cavity, which is not a common finding in adult population. Hydatid cyst rupture should be considered in the differential diagnosis of cases with pleural effusion. Rupture of a pulmonary hydatid cyst into the pleural cavity is rare, but represents the most serious complication of the hydatid disease. In regions where echinococcal disease is endemic, a high level of clinical suspicion is necessary for diagnosis and appropriate management of this condition.

*Corresponding author: Dr. Divya Rabindranath, Department of Pathology, J.N.Medical College, A.M.U., Aligarh, U.P., India. 202001. Phone: +91- 7895683197 E-mail: divy30@hotmail.com

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Rabindranath et al.

Introduction

Human Echinococcosis is a serious helminthic disease affecting endemic areas being Mediterraanean countries, South Africa ,Australia, New Zealand and Central Asia including India.[1] Most common form of disease found in humans is called as unilocular Echinococcosis caused by Echinococcus granulosus.[2] The next common being Alveolar Echinococcosis caused by Echinococcus multilocularis followed by Polycystic Hydatid disease caused by Echinococcus vogeli and rarely Echinococcus oligarthus. Disease can involve any part of the body but seen most commonly in liver and lungs. Manifestation of the disease depends on location in the body. Pulmonary Echinococcosis is seen rarely in adults and usually remains asymptomatic until the time of rupture. Rupture of pulmonary cyst into the pleural cavity is rare, but represents the most serious complication of the hydatid disease. The incidence of rupture is found to be more frequent in children and reported to be 26.7% in one series.[3] Ruptured hydatid cyst poses a diagnostic challenge as radiographic picture is highly varied. It is diagnosed usually by imaging, histological, cytopathological and serological techniques. Surgery with postoperative anthelminthic therapy is the treatment of choice, and a close follow-up is required.

C-171 adult population. Patient was started on albendazole 400mg twice a day for 6 cycles of 4 weeks each with 2 weeks drug free interval between the cycles since then and follow up was done every 3 monthly for 1 year with serial radiological and clinical examinations, during which the patient remained asymptomatic and showed no signs of recurrence.

Case Report

A 45 year old male came to our emergency department in shock with symptoms of shortness of breath and altered mental status from the previous day. There was no history suggestive of bronchial asthma, drug intake, allergy or chest trauma. His blood pressure was 80/60 mm Hg, heart rate 128 beats/min, respiratory rate 42 breaths/min, and temperature 38.5째C. Investigations revealed Hb of 11.1gm%, total leucocyte count of 19,280 cells /cumm, (P72, L17, M1, E10, B0). ESR was 110mm/hr. Radiograph showed massive left sided pleural effusion with right sided round well circumscribed non homogenous opacity suggestive of loculated pleural effusion (Fig.1).Therapeutic aspiration was done. Pleural fluid examination revealed total leucocyte count to be 550cells/cumm, and eosinophilia. Cytology showed hydatid scolices with neutrophils in the background (Fig.2). Immunoglobin-G enzyme-linked immunosorbent assay for Echinococcus was positive. CT scan revealed massive left sided pleural effusion with shift of mediastinum to contralateral side with basal pleural thickening and floating lily sign. Right lung also showed a round well circumscribed fluid filled cystic lesion with multiple septations suggestive of multiple daughter cysts and basal hydatid sand (Fig.3). Ultrasound abdomen was suggestive of normal study with no involvement in liver, thereby confirming the diagnosis of primary bilateral hydatid lung disease with rupture into the pleural cavity on the left side, which occurs rarely in the www.pacificejournals.com/apalm

Fig.1: Radiograph showing massive left sided pleural effusion with right sided round well circumscribed non homogenous opacity suggestive of loculated pleural effusion.

Fig. 2: Pleural fluid smear showing hydatid scolice with neutrophils in the background (H&E, 40x)

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Echinococcosis on Pleural Effusion Cytology pleural cavity may occur causing pleural effusion, simple or tension pneumothorax,[3] empyema and anaphylactic shock,[5] due to release of antigenic material as in our case. Other complications can be secondary bacterial infections or development of secondary lesions in surrounding tissues. Ruptured and infected hydatid cysts are often confused with pneumonia, tuberculosis, lung abscess, tumor, or pneumothorax.[3] But the radiological, cytological and serological tests can help us in confirming the diagnosis.

Fig. 3: CT scan showing massive left sided pleural effusion with shift of mediastinum to contralateral side with basal pleural thickening and floating lily sign (yellow arrow) and round well circumscribed fluid filled cystic lesion with multiple septations (red arrow) suggestive of multiple daughter cysts and basal hydatid sand on the right side.

Discussion

Cystic hydatid disease or Echinococcosis is a zoonotic disease affecting humans by ingestion of food or water contaminated with eggs of echinococcal species, or by direct contact with infected dogs or canines. Most common involved species is Echinococcus granulosus.[2] Rarely infection can be caused by Echinococcus multilocularis, Echinococcus oligarthus, Echinococcus vogeli. Hydatid disease is endemic in Eurasia, North East Africa, Australia and South America.[1] For Echinococcus granulosus, dogs act as definitive hosts which harbor adult tapeworm, and shed eggs containing larval stage (metacestode). Infection is transmitted to intermediate host (most commonly sheep) by ingestion of eggs and to humans as accidental intermediate hosts. Disease in man is characterised by benign slowly growing cysts most commonly seen in liver (50-70%), lungs (2030%), spleen, rarely in pleural cavity, kidney, spleen, bone, heart, CNS and other organs.[4] Pulmonary hydatidosis is rare in adults and usually asymptomatic, though patients may present with cough, dyspnea, hemoptysis due to mass effect of the cysts exerting pressure on surrounding tissues. Appearance of new symptoms or exaggeration of existing ones may follow complications of the disease. Most often complicated by cyst rupture leading to flow of larval tissue fragments and protoscolices into bronchial tree causing chest pain, fever and hemoptysis. Rupture into

Diagnostic interventions include imaging, histopathological, cytological examination and confirmation by serology. A spectrum of cytomorphological features of hydatidosis can be seen on FNAC smears including scolices, lamellated wall, hooklets, eosinophils and sometimes charcot laden crystals.[6] Present case was diagnosed with the help of FNAC, which is usually discouraged in suspected hydatid disease due to risk of anaphylaxis. On X-ray “meniscus sign”, spherical cyst, blunting of CP angle,[7] and on CT scan “water lily sign”, large cystic lobulated structures containing multiple daughter vesicles or membranes, septa, hydatid sand,[8] and recently discovered “air bubble sign” aids in establishing diagnosis of perforated pulmonary hydatid cyst, as also seen in our case. Release of parasitic antigens in presence of complications relate to positive serological diagnosis and supports clinical diagnosis of ruptured cystic echinococcosis. Quantitative serodiagnostic methods like Immunoglobulin G enzymelinked immunosorbent assay (ELISA) test with sensitivity of 85.3% can be used which are commercially available. [9] When feasible early surgery is the treatment of choice for pulmonary hydatidosis to prevent eventual rupture. In perforated cases medical therapy with albendazole is given as a complement to surgical treatment, to avoid recurrence, and in patients who cannot undergo surgery.[10]

Conclusion

We report this case of ruptured pulmonary hydatid cyst in pleural cavity for its unusual presentation, usually rare in adults and to emphasize the role of FNAC as an extremely useful, cost effective and safe tool in diagnosing the disease. Thus hydatid cyst rupture should be suspected in patients presenting as pleural effusion, from areas where Echinococcus is endemic, as it represents the most endangered life threatening complication of hydatid disease.

Acknowledgements nil

Funding none

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

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Competing Interests None declared

Reference

1. S Gole, G Gole, V Satyanarayana. Unusual Presentation of Hydatid Cyst: A Case Series With Review of Literature. The Internet Journal of Parasitic Diseases. 2013; 6 (1). 2. Goyal P, Ghosh S, Sehgal S, et al. Primary Multilocular Hydatid Cyst of Neck with Unique Presentation: A Rare Case Report and Literature Review. Head and Neck Pathology. 2014;8:334-338. 3. Balci AE, Eren N, Eren S, and Ulka R. Ruptured hydatid cysts of the lung in children: clinical review and results of surgery. Annals of Thoracic Surgery. 2002; 74 (3): 889-892. 4. Gottstein B, Reichen J. Hydatid lung disease (echinococcosis/hydatidosis). Clin. Chest Med. 2002; 23 (2): 397-408. 5. Aribas OK, Kanat F, Gormus N and Turk E. Pleural complications of hydatid disease. Journal of Thoracic and Cardiovascular Surgery. 2002; 123 (3): 492-497.

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C-173 6. Goyal P, Sehgal S, Ghosh S, Mittal D, Kumar A, Singh S. A Cytological Study of Palpable Superficial Nodules of Parasitic Origin: A Study of 41 Cases. Pathology Research International. 2014;2014:373472. doi:10.1155/2014/373472. 7. Rai SP, Panda BN, Ganguly D, Bharadwaj R. Pulmonary hydatid: diagnosis and response to hypertonic saline irrigation and albendazole. Med J Arm Forces India. 2005; 61 (1): 9-12. 8. Safioleas MC, Misiakos EP, Kouvaraki M, Stamatakos MK, Manti CP, Felekouras ES. Hydatid Disease of the Liver A Continuing Surgical Problem. Arch Surg. 2006; 141: 1101-8. 9. Niscigorska J, Sluzar T, Marczevska M, et al. Parasitic cysts of the liver:practical approach to diagnosis and differentiation. Med Sci Monit. 2001; 7: 737-41. 10. Fanne RA, Khamaisi M, Mevorach D, Leitersdorf E, Berkman N, Laxer U, et al. Spontaneous rupture of lung echinococcal cyst causing anaphylactic shock and respiratory distress syndrome. Thorax. 2006; 61: 550.

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Case Report Undifferentiated Subtype of Non-keratinizing Carcinoma of the Nasopharynx Metastasizing to the Breast: A Case Report and Literature Review Ali Koyuncuer* Department of Pathology, Antakya State Hospital, Hatay, Turkey Keywords: Breast, carcinoma, metastasis, nasopharyngeal carcinoma

ABSTRACT Nasopharyngeal carcinomas (NPCs) have a particularly high risk for aggressive clinical behavior. Metastatic tumors or secondary malignant neoplasms to the breast are uncommon. Breast metastasis of NPCs is very rare. We report a case of NPC in a 17-year-old female patient presenting with a mass in her left breast. The tumor consisted of an inflammatory infiltrate rich in lymphoplasmacyticcells and composed of carcinoma cells having large, vesicular, round to oval nuclei with single, centrally located, eosinophilic nucleolus with eosinophilic cytoplasm. The epithelial tumor cells were strongly positive for pancytokeratin and CK19 and negative for CK7, CK20, progesterone, and estrogen receptors. NPCs are predominantly seen in adults with an average age of 50 years at diagnosis, and they are slightly more common in men than in women. The etiology of NPCs is generally multifactorial, such as genetic predisposition, Epstein-Barr virus infection, and environmental factors. A review of the literature on NPC yielded eight cases of metastasis to the breast. Excessive radical surgery is not suitable for metastatic tumors in the breasts of these patients, but isolated success has been obtained with a combination of local surgery and radiation therapy.

*Corresponding author: Dr. Ali Koyuncuer, Pathologist, Department of Pathology, Antakya State Hospital, Hatay, Turkey. Phone: +91- 0905063819352 E-mail: alikoyuncuer@hotmail.com

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in diameter, and serial sections of the surgical specimen did not reveal tumor infiltration to the peripheral compartment. Its gross appearance was a soft, grayish-white tissue, which was a well-localized, well-demarcated, smooth mass. The cut surface of the tumor revealed ill-defined, solid areas that were focally prominent and gray with myxoid changes (Fig. 2). Microscopically, the tumor consisted of a prominent, chronic inflammatory infiltrate rich in denselymphoplasmacyticinfiltrate with less eosinophilic background and composed of carcinoma cells having large, vesicular, round-to-oval nuclei with single, centrally located eosinophilic nucleoli with eosinophilic cytoplasm. composed of sheets of cohesive epithelial cells with only small foci lacking cohesive tumor cells. Immunohistochemically, the epithelial tumor cells were diffusely and strongly positive for pancytokeratin and CK19. The cells were focally positive for CK5/6 but negative for CK7, CK20, progesterone receptors, and estrogen receptors (Fig. 3A, B, C, D,E). Absence of in situ breast (ductal or in situ lobular) carcinoma.

Metastatic tumors in the breast arise for the most part in malignant melanoma, lymphoma, lung cancer, and carcinoma of the ovary[1]. Metastases from the head and neck region from malignant tumors are uncommon [1]. For nasopharyngeal carcinomas (NPCs), the relationship between race, geographical areas, and multifactorial etiology should be noted. Worldwide, NPC constitutes approximately 65,000 new cases per year and accounts for 38,000 cancer cases death annually. The vast majority of neoplasms in this category are non-keratinizing carcinomas, which itself are the most common subtype of undifferentiated carcinomas [2]. Occasionally, patients with NPCs have presented with lymphadenopathy, and the most frequent metastatic sites are retropharyngeal and level II lymph nodes [3]. Distant metastases occur most often to bone, lung, liver, and retroperitoneal lymph nodes [4]. To the best of our knowledge, only eight cases have been reported previously in the medical literature [1, 5, 6, 7,8] (Table 1). The clinicoradiological findings and pathological features of this unusual presentation are reported here.

Case report

A 17-year-old female arrived at the surgery department of a state hospital with non-specific, systemic symptoms, such as breast pain and palpable breast mass was detected. Breast ultrasonography and upper abdomen computed tomography (CT) imaging revealed the presence of a 7.8 cm × 4.8 cm solid mass in the left breast region, suspicious for a neoplasm and therefore was resected completely. We report a case; which was diagnosed in 2011 in a patient, he was diagnosed with stage I (T1N0M0) nasopharyngeal carcinoma (NPC) with tumor confined to nasopharynx. Magnetic resonance imaging was performed on the nasopharynx posterior wall and lateral walls, following an injection of intravenous contrast. T1a weighted signal due to post-contrast scans are performed; hypointense and T2a weighted signal due to hyperintense. The patient was diagnosed with NPC and developed a left breast mass (Fig.1A, B, C, D).The tumor measured 4 cm × 1 cm × 1 cm

Discussion

The incidence of breast involvement by other nonmammary malignancies is very rare (approximately 0.4% to 1.3%). The most common sources of metastases to breast are primary neoplasms from adverse effects of breast lymphoma or leukemia and malignant melanoma [9]. First described by Dawson (1936), metastases, the stomach signet ring cell carcinoma, is situated at the lymphatic channel in the breast. With these characteristics, the median interval to the development of a breast metastasis is approximately 2 years for cases with previously treated cancer. Metastatic disease, defined as a single lesion in the breast region, is seen in approximately 85% of cases [10] . It is more common in Southern Chinese and African populations, in which the annual incidence rises to 30–50 per 100,000 persons [11] and 23.3 and 8.9 cases per 100,000 persons per year for men and women, respectively [4]. The age distribution is similar in men and women; however, it occurs predominantly in males [2]. Although the etiology is multifactorial, including genetic predisposition and specific

Table 1: Data of the published cases of NPC metastasis to the breast. References Year Sham [5,20] 1986 Sham[5,20] 1987 Driss [1] 1999 Que [19] 2003 Yeh [6] 2004 Vaishnav[7] 2012 Liang[8] 2013 Leach [5] 2013 This case 2013

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Age(years) 39 51 25 41 44 49 49 17

Gender F F F F F F M F F

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Table 2: Chronological history (treatment and diagnosis) of the the case. Treatment or diagnosis processes The initial presentation

Treatment:conventional radiotherapy

Breast metastasis

Diagnosis and treatment

Date

NPC

Stage

11.10.2011 Magnetic Stage I resonance imaging (T1N0M0) (MRI)

Head and neck region: -Radiotherapy: 43 days, total dose 70 Gy. -Chemotherapy: Cisplatin 178 mg/first day (100mg/m2), 5 Fluorouracil 1780 mg/5 day (1000 mg/ m2) every 21 days. NPC

Preoperative -Radiotherapy: 15 days, total dose 70 Gy Surgery:Pathologic Undifferentiated Subtype of Non-keratinizing diagnosis Carcinoma of the Nasopharynx After surgery Local excision, Postop. -Chemotherapy: Docetaxel 100 mg/ one day (60mg/m2), 5 Fluorouracil 2500 mg/2 day (1000 mg/m2), Carboplatin (AUC 5), four cycles, every 21 days. At the end of the 21-month The patient was disease-free and continuing to follow-up period come in for routine physical examinations.

Fig. 1: Magnetic resonance imaging (MRI). (A, B) Preoperative MRI. (A) Axial post-contrast T1-weighted MRI showing a nasopharyngeal carcinoma (B) Sagittal T1 weighted MRI. C,D; postoperative MRI and postoperative local radiotherapy. (C, D) Axial postcontrast T1-weighted MRI.

Radiology

19.06.2013 Computed Tomography (CT)

14.08.2013

Stage IVC (T1N0M1)

14.05.2015 MRI, CT

Fig. 2: Gross appearance of breast specimen.

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Fig. 3: (A) Undifferentiated nasopharyngeal carcinoma composed cells growth in a syncytial pattern and dense prominent lymphoplasmacytic infiltrate (H&E, 100×). Fig. 3: (B) Undifferentiated nasopharyngeal carcinoma composed of tumor cells of oval to round large vesicular nuclei, with eosinophilic nucleolus (H&E, 200×).

C-177 lymphatic spread [2]. Bones constitute the most common metastatic site (75%), followed by lung (46%), liver (38%), and retroperitoneal lymph nodes (10%) [4]. Previous studies demonstrate the mean age of breast metastases from undifferentiated NPCs is 42 years (25 to 51 years)(see Table 1). In the present case, we present the first 17-year-old case of breast metastases from NPC to expand the medical database; there are no previously described reports of this in the literature. NPCs can be subdivided into three distinct groups: non-keratinizing carcinoma (the most common form), undifferentiated subtype (WHO III), and the condition called lymphoepitheliomas [15]. Histologically, the undifferentiated subtype is characterized by solid sheets and nests of the epithelial cells with cytoplasmic eosinophilia and round or oval vesicular nuclei and large, central, and prominent nucleoli with a dense, prominent lymphoplasmacytic infiltrate [16].

carcinogens, by far the most important known predisposing factor is infection with the Epstein-Barr virus (EBV) [11, 12]. In this section, we present a simplified version, taking into account the etiopathogenetic correlation of precursor EBV and the undifferentiated form of NPC (WHO type III) [13]. NPCs are most common in the region of the lateral wall of the nasopharynx with particular frequency in the fossa of Rosenmüller. Grossly, most lesions present as exophytic, but it is rarely ulcerated. Patients may be asymptomatic or may present with a mass in the posterior cervical triangle or superior jugular chain of nodes and sometimes present in the otitis media, epistaxis, and nasal obstruction [14]. NPCs are generally characterized by more aggressive clinical behavior; most present with a metastasis with extensive locoregional involvement and hematogenous or

There are patterns of growth of NPC: Schmincke type is characterized by small clusters or aggregates and Regaud type also occurs in the large syncytial cells [17] .The immunophenotype of NPC has the following characteristics: positive for EBER, pancytokeratin, CK5/6, 34βE12, CK8, CD56, CK13, CK19, p63, and p53, but usually negative for CK4, CK7, CK10, CK14, CK20, p16, and HPV [14]. The most specific markers of breast carcinoma are gross cystic disease fluid protein 15 (GCDFP-15). There is apparently a diagnostically useful difference in the immunoprofile of breast carcinoma in the sense that the former is usually positive for CK7, GATA3, ER, and GCDFP-15 and negative for CK20 [18] . In most instances, the differential diagnosis of the undifferentiated subtype of non-keratinizing carcinoma resembles a number of other small round cell neoplasms of the sinonasal tract, including neuroblastoma, lymphoma, rhabdomyosarcoma, melanoma, plasmacytoma, and other [15] poorly differentiated carcinoma in primary breast cancer [10] .Radiotherapy for NPC, therefore, is the gold standard for treatment, and the 5-year survival rate in this series was 70% [11]. In contrast, the median survival for patients with metastatic tumors in the breast was approximately 10 months after the diagnosis of the metastases, and the patients ranged in survival from 0.4 to 192.7 months [10]. The prognosis of these metastatic tumors in the breast is substantially worse [7]. Primary treatment of metastatic tumors in the breast is radiation therapy and wide excision supplemented, if necessary, with therapy and/or combined axillary dissection. Mastectomy is not recommended as the therapy of choice [10]. The patient was treated with conventional radiotherapy andreceived radiotherapy to the

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Fig. 3: (C,D,E) Immunohistochemically positive for pancytokeratin, CK5/6 and CK19 (100×).

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head and neck region and during a follow-up appointment 20 months after the diagnosis of breast metastasis (Table 2). At the end of the 21-month follow-up period, the patient was disease-free and continuing to come in for routine physical examinations.

from extramammary malignancies. 2004;70:287-90 PMID: 15098776

Conclusion

In conclusion, breast metastases are most frequently confused with benign lesions and primary breast cancer. Obviously, metastasis to the breast requires a multidisciplinary approach with close cooperation and dialogue among the clinician, oncologist, radiologist, and pathologist. There should be careful comparison of suspicious foci with the cytologic or radiologic pattern in the initial diagnostic core needle biopsy. Therefore, patients with metastatic breast lesions may have been treated by unnecessary excessive radical surgery.

Conflict of interest

No financial competing interests

Funding source None

References

1. Driss M, Abid L, Mrad K, Dhouib R, Charfi L, Bouzaein A, et al. Breast metastases from undifferentiated nasopharyngeal carcinoma. Pathologica 2007;99:42830 PMID: 18416334 2. Chan JKC, Pilch BZ, Kuo TT, Wenig BM, Lee AWM. Tumours of the nasopharynx. In Barnes L, Eveson JW, Reichart P, Sidransky D. (Eds): Pathology and Genetics of Head and Neck Tumours, Lyon, IARC Press, 2005;81-106 3. Ho FC, Tham IW, Earnest A, Lee KM, Lu JJ. Patterns of regional lymph node metastasis of nasopharyngeal carcinoma: a meta-analysis of clinical evidence. BMC Cancer 2012;21:12:98 PMID: 22433671, doi: 10.1186/1471-2407-12-98. 4. Huang CJ, Leung SW, Lian SL, Wang CJ, Fang FM, Ho YH. Patterns of distant metastases in nasopharyngeal carcinoma. Kaohsiung J Med Sci 1996;12:229-34 PMID: 8683644 5. Leach BI, Sun B, Petrovic L, Liu SV. Breast metastasis from nasopharyngeal carcinoma: A case report and review of the literature. Oncol Lett 2013;5:1859-1861 PMID: 23833656, DOI: 10.3892/ol.2013.1303 6. Yeh CN, Lin CH, Chen MF. Clinical and ultrasonographic characteristics of breast metastases

Surg

7. Vaishnav KU, Pandhi S, Shah TS, Chaudhry A.Nasopharynx carcinoma: a rare primary for bilateral breast metastasis.BMJ Case Rep. 2012 Jul 9;2012. pii: bcr0320126083. doi: 10.1136/bcr.03.2012.6083. 8. Liang N, Xie J, Liu F, Xu D, Yu X, Tian Y, et al. Male breast metastases from nasopharyngeal carcinoma: A case report and literature review. Oncol Lett. 2014;7:1586-1588. Epub 2014 Feb 20. 9. Maounis N, Chorti M, Legaki S, Ellina E, Emmanouilidou A, Demonakou M, et al.Metastasis to the breast from an adenocarcinoma of the lung with extensive micropapillary component: a case report and review of the literature. Diagn Pathol 2010;17:5:82 PMID: 21167048, doi: 10.1186/1746-1596-5-82. 10. Hoda SA. Metastases in the Breast from Nonmammary Malignant Neoplasms. In Rosen PP (Ed): Rosenâ&#x20AC;&#x2122;s Breast Pathology, 4nd ed., Lippincott Williams&Wilkins, Philadelphia, 2014;937-956 11. Sidler D, Thum P, Winterhalder R, Huber G, Haerle SK. Undifferentiated carcinoma of nasopharyngeal type (UCNT): a Swiss single-institutional experience during 1990-2005. Swiss Med Wkly 2010;140:273-9 PMID: 19950040, doi: smw-12844. 12. Chu EA, Wu JM, Tunkel DE, Ishman SL. Nasopharyngeal carcinoma: the role of the EpsteinBarr virus. Medscape J Med 2008, 10:165 PMID: 18769688 13. Gullo C, Low WK, Teoh G. Association of EpsteinBarr virus with nasopharyngeal carcinoma and current status of development of cancer-derived cell lines. Ann Acad Med Singapore 2008;37:769-77 PMID: 18989494 14. Thompson LD. Update on nasopharyngeal carcinoma. Head Neck Pathol 2007;1:81-6 PMID: 20614287, doi: 10.1007/s12105-007-0012-7 15. Barnes L.Diseases of the Nasal Cavity, Paranasal Sinuses, and Nasopharynx. In Barnes (Ed): Surgical Pathology of the Head and Neck, New York, Informa Healthcare, 2008;343-423 16. Wakely PE.Nasopharengeal Carcinoma, Nonkeratinizing Undifferentiated Type. In Suster S (Ed): Head and Neck Pathology, New York, Demos Medical, 2011;17-18

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Koyuncuer et al. 17. Regauer S. Nasopharynx and Waldeyerâ&#x20AC;&#x2122;s Ring. In Cardesa A, Slootweg PJ (Eds.): Pathology of the Head and Neck, New York , Springer, 2006;171-190 18. Bhargava R, Dabbs DJ. Immunohistology of the Breast. In David J. Dabbs. (Ed): Diagnostic Immunohistochemistry Theranostic and Genomic Applications, Philadelphia, Elsevier Saunders, 2014;710-761

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C-179 19. Que J, Chien-Tai H, Shih-Sung C, Li-Ching L, Kue-Li L. Breast Metastases from Nasopharyngeal Carcinoma: a case report and literature review. Chin J Radiol 2003;28:323-327 20. Sham JS, Choy D. Breast metastasis from nasopharyngeal carcinoma. Eur J Surg Oncol. 1991;17:91â&#x2013;Ą93

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Case Report Hydatid Cyst in Femur: - A Case Report Shweta Chaturvedi*1, Shubha Gupta1, Mansi Faujdar1, Gajendra Gupta1, Jaimini Patel1, Vishal Khandelwal2 Department of Pathology, Santokba Durlabhji Memorial Hospital, Jaipur, India 2 Department of Pathology, JLN Medical College, Ajmer, India

Keywords: Echinococcus, Hydatid Cyst, Osseous hydatidosis, Femur

ABSTRACT Hydatid cyst is caused by the larva of Echinococcus granulosus. Its usual occurrence is in liver and lungs. Osseous hydatidosis is a rare event and is often a diagnostic challenge to the clinician. We report a case of 18 year old boy who presented with complaints of pain over left hip for past one year and subsequently whose MRI was done which suggested it the lesion to be either due to giant cell tumour or chondroblastoma. On the contrary, histopathological examination of the lesion stated it to be osseous hydatid cyst.

*Corresponding author: Dr Shweta Chaturvedi, Department of Pathology, Santokba Durlabhji Memorial Hospital, Jaipur, India

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sent for histopathological examination which revealed a cystic lesion, with lamellated cyst wall and multiple scolex attached to the cyst wall (fig.2 & fig.3); thereby giving the final impression as hydatid cyst.

Hydatidosis is an infectious disease caused by the parasitic tapeworm Echinococcus. The species most responsible for hydatid disease is Echinococcus granulosus, which is endemic especially in cattle raising countries. Liver (55-70%) is the most common site of hydatid disease followed by the lungs (18-35%). Bone involvement is very rare accounting for only less than 1% of the patients [1,2], yet it is the most debilitating form of echinococcosis in humans. Intra-osseous lesions usually begin at the epiphysis and causes bone destruction through three mechanisms: compression, ischemia and osteoclast proliferation around the compressed bone tissue causing thinning and fracture and extension into soft tissues [1]. The aim of our study is to spread awareness about the occurrence of osseous hydatid cyst so that prompt treatment could be individualized according to the site, extent and severity of involvement.

Case Report

An 18 year old male patient presented with complaints of pain over left hip for the past one year which increased on walking and weight bearing. Clinically there was tenderness over left hip. X- Ray revealed an osteolytic lesion over neck and head of femur (fig.1).

Fig. 2: Microscopy revealing lamellated cyst wall and attached scolices (H&E, 40x)

Fig. 3: Higher magnification of scolex (H&E X 100).

Fig. 1: Plain radiograph showing lytic lesion over neck and head of left femur

Further , MRI of hip joints was done which showed cystic / near fluid intensity areas in left femoral head and neck superolaterally with evidence of severe cortical thinning or breach along the inferolateral aspect of femoral head and superolateral aspect of neck suggesting a differential diagnosis of Giant cell tumour or chondroblastoma.

A sample of the lesion was also sent for microbiological examination and smears were prepared and stained with Gram Stain and Z-N stain. Gram stained smears showed few scolex of hydatid cyst while the smear of Z-N stain showed no acid fast bacilli. USG scan of abdomen was normal as well as SGOT/SGPT tests were within normal limits. Also to be noted that, Elisa test for detecting anti â&#x20AC;&#x201C; Echinococcus studies was negative.

Hematological investigations showed mild eosinophilia, normocytic normochromic RBC(s) and an ESR of 8mm/ hr. A Biopsy of the lesion over head of femur was done and

Patient was given a course of albendazole to subside the parasitic infection and and after that surgical resection of the lesion was advised. After taking the prescribed treatment, he was operated and complete debridement of the lesion was done and the sample was sent for the

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histopathological examination. Biopsy revealed fragments of mature bony tissue, lymphoplasmacytic cells and few foreign body giant cells. Also seen were fragments of a cyst wall showing lamellations susggesting the possibility of hydatid cyst.

Discussion

Hydatid cysts caused by Echinococcus species can produce tissue cysts anywhere in the body. Skeletal involvement is rare, yet because of their unusual presentation diagnosis can be missed. Primary hydatid disease of the bone is infrequent because the parasites must pass the filters of the liver and lungs. The disease manifests itself differently in the bone due to the mechanical resistance that the bone offers to the growth of parasites and cysts. The lesion tends to present with pain or pathological fracture and occur in the thirty to fifty year old age group [3]. But in our case the patient was young. The diagnosis of osseous hydatidosis is difficult and is easily overlooked unless there is a strong element of suspicion [4] . It may mimic tuberculosis, chronic osteomyelitis, simple bone cyst, sub-acute arthtritis, giant cell tumours, osteosarcoma, malignant fibrous histiocytoma, myeloma and chondrosarcoma. Plain skiagram, CT, MRI remains the common modes of diagnostic tools. Fine- needle aspiration must be avoided because of the danger of spread, sensitization, and anaphylaxis [4,5,6]. However, despite all investigative measures, diagnosis is established only at surgery or biopsy of the lesions. [7], [8], [9] The cyst microscopy shows cyst wall comprising of three layers. The innermost is the germinal layer which gives rise to brood capsules that are attached to the inner wall by a short stalk [10]. Protoscolices develop within the brood capsule and when they detach they are called daughter cysts. Cyst fluid content obtained at FNA consists of daughter cysts protoscolices. Second layer is the ectocyst which shows a laminated membrane that is avascular, eosinophillic, refractile and chitinous.The third layer is host derived and known as pericyst. It consists of dense fibrovascular tissue with inflammatory cells. Hydatid disaese in bone should be treated with radical resection, with a wide margin of the healthy tissue. This may be difficult, but incomplete removal results in recurrence. [4]

Conclusion

The purpose of this article is to emphasize the fact that this disease should be suspected in cystic lesions affecting the bone, so that early diagnosis helps in eradication and salvage of the bone. Misdiagnosis and delayed diagnoses are always fraught with the danger of amputation, recurrence and sepsis.

Acknowledgements nil

Funding none

Competing Interests None declared

References

1. Zlitini M, Ezzaoula K, Lebib H, Karray M, Kooli M, Mesteri M. Hydatid cyst of bone: diagnosis and treatment. World J Surg.2001;25:75–82 2. Szypryt EP, Morris DL, Mulholland RC. Combined chemotherapy and surgery for hydatid cyst of bones. J Bone Joint Surg Br.1987;69:141–4 3. Khandakar HH, Islam S, Begum B A, Khatun M, Alam M A. Hydatid disease in femur:A case report. Dinajpur Med Col J 2012;5:56-7. 4. Herrera A, Martinez AA. Extraspinal bone hydatidosis. J Bone Joint Surg Am. 2003;85 :1790–4. 5. Sapkas GS, Stathakopoulos DP, Babis GC, Tsarouchas JK. Hydatid disease of bones and joints.8 cases followed for 4–16 years. Acta Orthop Scand.1998;69:89-94 6. Papanikolaou A. Osseous hydatid disease. Trans R Soc Trop Med Hyg. 2008; 102: 233–8. 7. Tomak Y, Dabak N, Gulman B, Karaismailoglu TN, Basoglu T, Incesu L. Hydatid disease of the left femur: a case report. Bull Hosp Jt Dis. 2001-2002; 60:89-93. 8. Tuzun M, Hekimoglu B. CT findings in skeletal echinococcosis. Acta Radiol. 2002;43:533-8. 9. Yildiz K, Gurbuz Y, Buluc L, Cirpici Y. Diagnostic clues in fine needle aspiration biopsy of hydatid disease of bone. Acta Cytol. 2006; 50:353. 10. Goyal P, Ghosh S, Sehgal S, et al. Primary Multilocular Hydatid Cyst of Neck with Unique Presentation: A Rare Case Report and Literature Review. Head and Neck Pathology. 2014;8:334-8.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Case Report Gigantic Lipoleiomyoma of Cervix with Extensive Hyaline Degeneration: A Case Report with Review of Literature Rupali Mandal, Krishnendu Mondal*, Pulakesh Pramanik Department of Pathology, Dishari Healthpoint, Malda, West Bengal, India Keywords: Adipocytes, Cervix, Lipoleiomyoma, Postmenopausal female, Smooth muscle.

ABSTRACT Uterine lipoleiomyomas are rare benign neoplasms of mature smooth muscle and adipose tissue. Although mostly located intramurally within uterine corpus; cervix is rather an unusual site of its origin. Premenopausal or postmenopausal obese ladies commonly suffer with this tumour. Lipoleiomyomas generally grow asymptomatically, but larger tumours often elicit symptoms due to pressure effects exerted on adjacent organs. We highlight a giant cervical subserosal lipoleiomyoma in a 48-years-aged menopausal female. She presented with ill-defined lower abdominal pain. Radiologically a circumscribed genital mass was detected, which was removed with total abdominal hysterectomy plus bilateral adnexectomy.

*Corresponding author: Dr Krishnendu Mondal, Vill- Fularhat, P.O. & P.S. Sonarpur, Dist- South 24 Parganas, 700150, West Bengal, India. Phone: +91- 9836740602 E-mail: krishnendu.kriss@gmail.com

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Gigantic Cervical Lipoleiomyoma

Introduction

Lipoleiomyoma, an uncommon benign mesenchymal neoplasm, represents 0.03-2.9% of all leiomyomas arising at female genital organs.[1-5] Uterine corpus is its usual site of origin. But, around 6.5-14% of lipoleiomyomas originate in cervix. Broad ligament, peritoneum and ovary are the other secluded locations.[3-5] It is mostly sited intramurally while about 13-20% tumours grow subserosally. Asymptomatic in its evolution, lipoleiomyomas are generally discovered incidentally on surgical specimens.[4,5] Mean diameter of lipoleiomyomas range from 4.6-5.5 cm.[3,4] Obesity and peri- or postmenopausal age-groups have highest association with this neoplasm. Structurally, it is characterized by an implicit cohabitation of mature smooth muscle fibres and adipose tissue in variable proportions.[6] Compared to pure leiomyomas, degenerative changes rarely appear in lipoleiomyomas.[4] This report explores a huge subserosal lipoleiomyoma with extensive hyaline degeneration, originating from the cervix of a 48-years-old postmenopausal female.

Case Report

A 48-years-aged, P2+0, three-years-postmenopausal lady attended the gynaecologic out-patients clinic with vague, poorly-localized, lower abdominal pain for past four months. She did not complain about any definite gastrointestinal or genitourinary symptomatic. Even her per-vaginal and per-rectal examinations proved to be disappointing as well.

Microscopically, the tumour was well-delineated and pseudo-encapsulated. It was comprised of uniform spindle-shaped, mature smooth muscle cells arranged in intersecting fascicles; with interspersed benign adipocytes in small groups and singles. The myocytes featured monomorphic elongate cigar-shaped nuclei, fine chromatin, inconspicuous nucleoli and moderateto-abundant amount of eosinophilic fibrillary cytoplasm (Figure 2). The myogenic component was often replaced by diffuse extracellular collagenous deposits, with minimal alteration in the adipocytic topography (Figure 3). Exuberant cytological atypia or mitosis, necrosis and calcification were imperceptible. Ultimately, the tumour was confidently diagnosed as ‘cervical lipoleiomyoma with extensive hyaline degeneration’. Following an uneventful postoperative outcome, the patient recovered satisfactorily during next six months’ follow-up.

Discussion

Lipoleiomyoma is an extremely uncommon uterine fatty tumour, regarded as a histologic subtype of leiomyoma. Collectively it constitutes less than three per cent of all uterine leiomyomas.[2-5] That is why its true incidence among general population is yet elusive.[5] Among the handful of case series published so far, Akbulut et al.[4] examined maximum number of subjects i.e. 76 cases; followed by Wang et al.[3] (50 cases), Aung et al.[2] (17 cases) and Bolat et al.[5] (10 cases). The histogenesis and nomenclature of ‘lipoleiomyoma’ has long been debated. Previous erroneous theorems

Next step she underwent abdomino-pelvic ultrasound; which identified a massive heteroechoic mass involving her genital organs, measuring approximately 18×17 cm. Sonographic discrimination between uterine or salpingooophoritic echotexture was impossible. Under this obscure clinico-radiologic circumstance, the lady was scheduled for total abdominal hysterectomy with bilateral salpingooophorectomy and accordingly the tumour was en masse despatched for histopathological examination. Grossly a circumscribed, gigantic, multilobulated, subserosal neoplastic mass; spanning 19×18×16 cm, was seen to be circumferentially emanating from cervix; and extended upwards to squeeze the corpus in between tumoural lobules. Its excised surface appeared solidhomogeneous, greyish-white, punctated with paleyellowish foci. Haemorrhage, necrosis, or calcification was absent. Morphologically, rest of the genitalia maintained its normal architecture (Figure 1). Provisionally, a diagnosis of ‘leiomyoma’ was strongly deliberated.

Fig. 1: Cervical lipoleiomyoma: Grossly, [inset] Circumscribed large mass arising subserosally from cervix, compressing the morphologically uninvolved uterine corpus and bilateral tubo-ovaries (red arrowheads); Cut surface homogeneous grey-white studded with yellowish-pale foci (black arrow-heads).

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C-185 The mean age of patients from the series of Aung et al.[2], Wang et al.[3] and Akbulut et al.[4] were 59.9, 53.9 and 55.49 years respectively. But, rarely it has also been reported in premenopausal youngers.[6,10] Lipoleiomyomas commonly arise intramurally within uterine corpus, while the submucosal origination is most unusual.[4] Cervix has been the site of origin in 7/50, 1/10 and 5/76 cases from the respective observations by Wang et al.[3], Bolat et al.[5] and Akbulut et al.[4]. Other uncommon locations include broad ligament[3,11] and retroperitoneum.[3,12] The discussed 48-years-old lady presented with a cervical subserosal lipoleiomyoma, which radiologically simulated a genital mass without any indication towards exact site of origin.

Fig. 2: Cervical lipoleiomyoma: Microscopically, intersecting fascicles of smooth muscle cells with intervening adipocytes in singles as well as in groups [H&E, 100x]; [inset] The myocytes have uniform cigarshaped nuclei, fine chromatin, inconspicuous nucleoli and moderate-to-abundant amount of eosinophilic fibrillary cytoplasm [H&E, 400x].

Fig. 3: Cervical lipoleiomyoma with hyaline degeneration: Replacement of the myogenic component by dense collagenous deposits [H&E, 100x].

Uterine lipoleiomyomas are generally asymptomatic, discovered incidentally on surgical specimens. Sometimes it evolve with pain, bleeding, mass, urinary frequency or a leiomyoma-like pressure-sensation.[2,3,8] Accordingly, the discussed patient complained about abdomino-pelvic pain at presentation. The diameter of lipoleiomyomas hardly exceeds 6 cm. In this respect Kim et al.[11] and Kalyankar et al.[10] notified two exceptionally large tumours, measuring beyond 15 cm. Such an extraordinarily huge mass was again encountered in present case. Lipoleiomyomas usually present as solitary lesion, while multifocality being a rarity. Moreover cervical fibroids may often enclose the cervix, simultaneously compress and displace the corpus superiorly. Such cases symptomatize from mechanical pressure exerted on adjacent organs.[13,14] Similarly, the lipoleiomyoma in discussed patient developed circumferentially from cervix, extended upwards to squeeze uterus and subsequently caused pain symptoms. Microscopically, lipoleiomyomas harbour a variable admixture of muscular and fatty components. Akbulut et al. subjectively classified it into grades I-III; as higher grades correspond to adipocytic abundance.[4] Likewise in present case, the lipomatous component was outnumbered by myogenic tissue, corresponding to grade I lipoleiomyoma. Normally necro-degenerative changes sparsely involve lipoleiomyomas. But similar to discussed case, Bolat et al. also detected prominent hyaline degeneration within three such lesions.[5]

like embryonic adipocyte misplacement, choristoma, hamartoma, or lipomatous degeneration, is currently obsolete. Nowadays, most researchers opine in favour of its metaplastic derivation from leiomyomas.[4] Back in 1916, detailed histopathology of lipoleiomyoma was first highlighted by Lopstein.[7] But, the term ‘lipoleiomyoma’ was popularized decades later, by Willen et al.[7] and Pounder[8], when they classified the uterine fatty tumours into ‘lipoma’ and ‘mixed lipoma/leiomyoma’ i.e. lipoleiomyoma. Moreover, Havel et al. demonstrated definite monoclonality in these tumours. Altogether these evidences substantiate its true neoplastic nature.[9]

Immunohistochemical (IHC) expression of vimentin, desmin, S-100, ER, PR and Ki-67 in lipoleiomyomas, has been implicated by several researchers to support its metaplastic histogenesis. But diagnostic implementation of IHC, has no extra advantage over routine haematoxylineosin stained method.[4,5] That’s for IHC was not considered in this reported case.

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Histological differential diagnoses of lipoleiomyomas include: spindle cell lipoma, angiolipoma, angiomyolipoma,

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and well-differentiated liposarcoma.[4,5] However, the absence of lipoblasts, atypia, necrosis or mitotic activity; paucity of angiomatous elements; and its characteristic clinicopathological presentation virtually clinched the diagnosis of lipoleiomyoma in present case.

Conclusion

Cervical lipoleiomyoma with extensive hyaline degeneration is a rare benign entity. Perimenopausal and postmenopausal women are most commonly affected. Although usually asymptomatic, larger tumours frequently elicit pain symptoms. Moreover, huge size often creates confusion on ultrasonography regarding its source organ. However, this dilemma is easily settled intraoperatively and final diagnosis can be confidently rendered on the basis of histopathological examination alone.

Acknowledgements

8. 9.

Nil

Funding

10.

None

Competing Interests None declared

11.

References

1. Prieto A, Crespo C, Pardo A, Docal I, Calzada J, Alonso P. Uterine lipoleiomyomas: US and CT findings. Abdom Imaging 2000; 25: 655-7. 2. Aung T, Goto M, Nomoto M, Kitajima S, Douchi T, Yoshinaga M et al. Uterine lipoleiomyoma: A histopathological review of 17 cases. Pathol Int 2004; 54: 751-8. 3. Wang X, Kumar D, Seidman JD. Uterine lipoleiomyomas: A clinicopathologic study of 50 cases. Int J Gynecol Pathol 2006; 25: 239-42. 4. Akbulut M, Gündoğan M, Yörükoğlu A. Clinical and Pathological Features of Lipoleiomyoma of the

12.

13.

14.

Uterine Corpus: A Review of 76 Cases. Balkan Med J 2014; 31: 224-9. Bolat F, Kayaselçuk F, Canpolat T, Erkanlı S, Tuncer I. Histogenesis of lipomatous component in uterine lipoleiomyomas. Turk Patoloji Derg 2007; 23: 82-6. Goyal P, Agrawal D, Ghosh S, Sehgal S, Kumar A, Singh S. Giant lipoleiomyoma of Cervix mimicking ovarian cancer in premenopausal woman: a rare case report and literature review. Journal of Gynecologic Surgery 2014; 30:24-7. Willen R, Gad A, Willen H. Lipomatous lesions of the uterus. Virchows Arch A Pathol Anat Histopathol 1978; 377: 351-61. Pounder DJ. Fatty tumours of the uterus. J Clin Pathol 1982; 35: 1380-3. Havel G, Wedell B, Dahlenfor R, Mark J. Cytogenetic relationship between uterine lipoleiomyomas and typical leiomyomas. Virchows Archiv B Cell Pathol 1989; 57: 77-9. Kalyankar V, Kalyankar B. Rare case of cervical lipoleiomyoma. J of Evolution of Med and Dent Sci 2014; 3: 5529-33. Kim HK, Kim JH, Hong SY, Choi YS, Oh HK, Lee TS. A uterine lipoleiomyoma of the broad ligament mimicking an ovarian tumor. Korean J Obstet Gynecol 2012; 55: 787-90. Schindl M, Birner P, Losch A, Breitenecker G, Joura EA. Preperitoneal lipoleiomyoma of the abdominal wall in a postmenopausal woman. Maturitas 2000; 37: 33-6. Fagouri H, Hafidi MR, Guelzim K, Hakimi I, Kouach J, Moussaoui DR, et al. Lipoleiomyoma of the uterine cervix(about an observation). International journal of scientific & technology research 2014; 3: 449-50. Agrawal D, Fotedar S, Daral R, Kumar K. Cervical lipoleiomyoma in premenopausal woman: a rare case report. Annals of Pathology and Laboratory Medicine, 2014;1, C14-17.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Case Report Congenital Hepatic Fibrosis Associated with Polycystic Kidney Disease Jaimini Natvarlal Patel*, Shubha Gupta, Gajendra Gupta, Mansi Faujdar, Shweta Chaturvedi Department of pathology, Santokba Durlabhji Memorial Hospital, Jaipur, India. Keywords: Congenital Hepatic Fibrosis, Portal hypertension, Celiac Disease, Polycystic Kidney Disease.

ABSTRACT Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder and occurs as a result of ductal plate malformation. Clinically it is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. The exact incidence and prevalence of CHF are not known, but it is a rare disease. This disorder is diagnosed in most patients during childhood or young adulthood. We present the case of 8 year old female with hepatosplenomegaly, hematemesis, melena, bilateral polycystic kidney disease and a histopathological diagnosis of congenital hepatic fibrosis. She had a history of celiac disease. Congenital hepatic fibrosis belongs to the so-called fibropolycystic diseases. Celiac disease is an immunemediated enteropathy. We describe its association with congenital hepatic fibrosis.

*Corresponding author: Dr Jaimini Natvarlal Patel, DNB Pathology, Santokba Durlabhji Memorial Hospital, Jaipur-302015, India. E-mail: Jaimini1988bd@gmail.com

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Congenital Hepatic Fibrosis

Introduction

Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. The exact incidence and prevalence of CHF are not known, but it is a rare disease. By 1988, only 200 patients with CHF had been reported in the literature. [1] The disease appears in both sporadic (in many as 56% cases) and familial patterns. Congenital hepatic fibrosis-ARPKD is estimated to occur in 1 in 20,000 live births. [2] Congenital hepatic fibrosis occurs as a result of ductal plate malformation. The ductal plate is a cylindrical layer of cells that surround a branch of the portal vein, and is the embryonic precursor of the intrahepatic bile ducts, as both interlobular and intralobular bile ductules develop from the ductal plate. Progressive remodeling starts at 12-week of gestation, and full maturation is usually complete by 20 week. Arrest of maturation and the lack of remodeling of the ductal plate that occurs as a result leads to the persistence of an excess number of immature embryonic duct structures. This abnormality has been termed the ductal plate malformation. The persistence of these immature duct elements stimulates the formation of portal fibrous tissue, and it is this periportal fibrosis that contributes to the clinical picture of recurrent cholangitis or portal hypertension and associated symptoms [3]. Clinically it is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. [1] The clinical manifestations of CHF are, however, nonspecific, making the diagnosis of this disorder extremely difficult. Onset of symptoms and signs is highly variable and ranges from early childhood to the 5th or 6th decade of life, although this disorder is diagnosed in most patients during adolescence or young adulthood [4]. The late appearance of symptoms and their clinical evolution suggest that CHF is a dynamic and progressive condition. We present it association with celiac disease.

failure or icterus. Temperature, pulse and BP were normal. On Abdominal examination, Liver measured 8cm below costal margin in epigastrium and Spleen measured 5cm below costal margin with tip above umbilicus. On Investigations, hemoglobin was 7g/dl, Total leucocyte count was 4.6Ă&#x2014;103 /Âľl, Platelet count was 1.5lakh/mm3. Liver function tests revealed total bilirubin was 1.8 mg/dl, AST was 56 IU/L, ALT was 59 IU/L and PT INR was 1.06. Ultrasound of Abdomen showed Hepatosplenomegaly with Portal Hypertensive changes, extensive periportal fibrosis with thinned out hepatic vein, bilateral Polycystic Kidney Disease, mild ascites. USG Doppler showed normal hepatic vein. Liver biopsy showed cores of liver tissue showing partial loss of normal lobe of architecture of liver. The portal tracts are markedly expanded due to large number of anastoming biliary channels lined by cuboidal epithelium, some of these channels are contact with parenchymal limiting plate. (Figure: 1) The channels are dilated and surrounded by fibrous tissue. The liver parenchyma is nodular as the result of surrounding fibrous bands. (Figure: 2, 3) Lumen of some of the ducts contains bile casts. (Figure: 4) The hepatocytes within nodules appear to be unremarkable. (Figure: 3, 5) There is no inflammatory reaction in the portal tracts. There is no evidence of steatosis. Histology is consistent with Congenital Hepatic Fibrosis.

Discussion

CHF is one of the fibropolycystic diseases, which also include Caroli disease, autosomal dominant polycystic kidney disease (ADPKD), and autosomal recessive polycystic kidney disease (ARPKD) [1].

Case Report

The 8 year old girl child was presented with abdominal distension for 2 years, history of hematemesis and melena 2 times in her life (at 6, 7 years of age). There was a history of celiac disease for 3 year. There was no history of abdominal pain or any skin bleeds and any hyperpigmentations. On tracing the pedigree, no other family member was known to be affected. She had no family history of renal cystic disease. She had a history of consangunity grade 3. General Examination revealed pallor without any sign of liver cell

Fig. 1: liver biopsy shows portal fibrosis and dilated bile channels (H&E stain; 40X)

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Fig. 2: Liver tissue showing portal fibrosis. (Massonâ&#x20AC;&#x2122;s trichrome stain; 40X)

Fig. 3: Bile channels are dilated and surrounded by fibrous tissue. The hepatocytes within nodule appear to be unremarkable (H&E stain; 100X)

Fig. 4: Lumen of some of ducts contains bile casts (H&E stain; 100X)

Fig. 5: Liver tissue showing normal hepatocyte arrangement within nodule (Reticulin stain; 40X)

Blyth and Ockenden have divided their patients into 4 groups called perinatal, neonatal, infantile and juvenile in accordance with the age at clinical presentation. Renal involvement is maximal in perinatal group and minimal in juvenile group [5]. Our patient had presented with hematemesis and melena (symptoms of Portal Hypertension), with no clinical or histological evidence of cholangitis and belongs to juvenile groups of this classification. CHF has initial presentation may be at around 3-6 months. The presentation ranges between 1.814 years [6], Portal Hypertension is a usual accompaniment and renal involvement is seen with < 10% tubules being affected. Classically affected patients are asymptomatic until the age of 5 or 7 years when manifestations of Portal Hypertension or cholangitis lead to the diagnosis. Several clinical forms are described which depend on the variable predominance of Portal Hypertension and cholangitis. Cholangitis form of CHF is more severe and usually occurs in late childhood and adult life.[1]

The usual presentation of CHF is with abdominal distension [7] , hematemesis or melena, failure to thrive, jaundice, anemia, hepatomegaly and splenomegaly [6, 8]. The other features of CHF are abdominal pain (splenic infarction), fever (cholangitis in dilated ductules), ascites, etc. [7, 9-11]. CHF is particularly associated with infantile polycystic kidney disease or intrahepatic bile duct dilatation (Caroliâ&#x20AC;&#x2122;s disease) [8]. Our case was associated with autosomal recessive polycystic kidney disease because of absence of parental history of renal cystic disease.

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An unequivocal diagnosis of congenital hepatic fibrosis can only be made by an examination of a liver biopsy. The classical histological findings of this disorder are varying degrees of hepatic fibrosis with nodular formation, which may become extensive as the disease progresses. In the eyes of inexperienced pathologists, histopathological findings may easily be mistaken for cirrhosis. In CHF, widened fibrous bands may be encountered in the portal

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tract containing an increased number of irregularly shaped proliferating bile ducts lined by normal cuboidal epithelium. Unlike cirrhosis, hepatic lobules are usually normal with normal hepatocyte morphology, particularly in the early stages. Other findings include cystic dilatation of bile ducts (Caroli’s disease), and hypoplasia of the portal vein branches in association with supernumerous hepatic artery branches. In fact, congenital absence of the portal vein has been reported in a pediatric patient with CHF. [12] The cholangitis form of CHF is difficult to differentiate from Caroli’s disease characterized by nonobstructive dilatation of intrahepatic bile ducts occurring as an isolated abnormality without portal fibrosis. Overlapping of CHF and Caroli’s disease has been confirmed by histological studies. [1] Celiac disease is an immune-mediated enteropathy triggered by the ingestion of gluten containing cereals, such as wheat, rye, or barley, in genetically predisposed individuals. Celiac disease is a common disorder, with an estimated prevalence of 0.5% to 1%. Biopsy specimens from the second portion of the duodenum or proximal jejunum, which are exposed to the highest concentrations of dietary gluten, are generally diagnostic in celiac disease. Pediatric celiac disease, which affects males and females equally, may present with malabsorption or atypical symptoms affecting almost any organ. Disease typically begins between ages of 6 and 24 months, after introduction of gluten to the diet, and includes irritability, abdominal distention, anorexia, chronic diarrhea, failure to thrive, weight loss, or muscle wasting. [13] Noninvasive serologic tests are generally performed prior to biopsy. The most sensitive tests are the presence of IgA antibodies to tissue transglutaminase or IgA or IgG antibodies to deamidated gliadin. [14] In a post-transplant group, 4.3% had celiac disease discovered before or after liver transplant. In this group, liver diseases recorded were primary biliary cirrhosis, autoimmune hepatitis, primary sclerosing cholangitis, and congenital hepatic fibrosis.[15] The mechanisms underlying liver injury in celiac disease are poorly defined. One probable mechanism is autoimmunity and genetic pre-disposition. [16]

Conclusion

In conclusion, we believe that knowledge on the association between CHF and celiac disease is relevant for a better diagnosis, treatment, and follow up of patients involved. As yet, no treatment modality has been shown to actually stop or even reverse the pathological process in

congenital hepatic fibrosis, and it remains a progressive and debilitating condition. The management and prognosis of CHF is dependent on alimentary bleeding secondary to Portal Hypertension. However, prognosis may be greatly improved by shunt surgery but survival in some patients may be limited by degree of renal failure.

References

1. De Vos M, Barbier F, Cuvelier C. Congenital hepatic fibrosis. J Hepatol 1988; 6: 222-228. 2. Turkbey B, Ocak I, Daryanani K, Esperanza FontMontgomery, Linda Lukose, Joy Bryant, et al. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol. 2009; 39:100-11. 3. Yonem O, Bayraktar Y. Is portal vein cavernous transformation a component of congenital hepatic fibrosis? World J Gastroenterol 2007; 13: 1928-1929. 4. Zeitoun D, Brancatelli G, Colombat M, Federle MP, Valla D, Wu T, et al. Congenital hepatic fibrosis: CT findings in 18 adults. Radiology 2004; 231: 109-116. 5. Blyth M, Ockenden BG. Polycystic disease of kidneys and liver. J Med Genet 1971; 8:257-84. 6. Summefield JA, Nagafuchi Y, Sherlock S, Cadafalch J, Scheuer PJ. Hepatobiliary fibropolycystic disease. A clinical and histological review of 51 patients. J Hepatol 1986; 2:141-156. 7. Abdullah AM, Nazer H. Congenital hepatic fibrosis in Saudi Arabia. J Trop Pediatr 1991; 37: 240-3. 8. Mowat AP. Congenital hepatic fibrosis. In: Liver Disorders in Childhood, 3rd edn. London, Butterworth Heinemam, 1993; pp 307-12. 9. Perisic VN. Long term studies on congenital hepatic fibrosis in children. Acta Paediatr 1995; 84: 695-6. 10. Ramiriz-Mayans JA. Congenital hepatic fibrosisStudy of 26 cases. Acta Gastroenterol 1994; 25: 297303. 11. Alvarez F, Bernard O, Brunelle F, Hadchowel H, Leblanc A, Odiewae M, et al. Congenital hepatic fibrosis in children. J Pediatr 1981; 99: 370-5. 12. Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol 2007; 37: 920-4. 13. Fasano A: Clinical presentation of celiac disease in the pediatric population. Gastroenterology 2005; 128:S68.

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14. Rostom A, Murray JA, Kagnoff MF. American Gastroenterological Association (AGA) Institute technical review on the diagnosis and management of celiac disease. Gastroenterology 2006; 131:1981. 15. Stevens FM, McLoughlin RM. Is celiac disease a potentially treatable cause of liver failure? Eur J Gastroenterol Hepatol. 2005;17:1015–7.

16. Azzam R. Liver Abnormalities in celiac Disease. Impact, A publication of the University of Chicago Celiac Disease Center; Summer 2010. www.cureceliacdisease.org/wp-content/ uploads/2011/09/0610CeliacCtr_Newslink.pdf (assessed on 19/11/2014)

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Case Report Low-Grade Appendicial Mucinous Neoplasm Presenting with Obstructed Inguinal Hernia: A Rare Association Abhishek Chowdhury*, Keya Basu, Uttara Chatterjee, Chhanda Datta, P.K. Sarkar, Manoj Kumar Choudhuri Institute of Post Graduate Medical Education & Research and SSKM Hospital, Kolkata, India. Keywords: Appendix, Adenocarcinoma, Pseudomyxoma Peritonei, Inguinal Hernia

ABSTRACT Low grade appendicial mucinous neoplasm is a rare entity. It is grouped under appendicial adenocarcinoma. Though it presents commonly with pain abdomen, asymptomatic patients are not uncommon. It is a low grade neoplasm, but capable of producing progressive and fatal disease in the form of pseudomyxoma peritonei. Management in case of localised appendicial disease includes simple appendectomy. We came across a patient presenting with obstructed inguinal hernia. While performing emergency exploration, a dilated appendix was uncovered. Appendectomy was done as a result and the histopathological examination of the appendix revealed that it was a low grade appendicial mucinous neoplasm.

*Corresponding author: Dr. Abhishek Chowdhury, 585/A/419/2, Kamalini Villa, DMS College Gate, Kaushallya, Kharagpur- 721301, West Bengal, India Phone: +91- 9477463948 E-mail: abhishekchowdhury26@yahoo.com

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Chowdhury et al.

Introduction

Low -grade Appendicial Mucinous Neoplasm (LAMN) is a rare entity grouped under appendicial adenocarcinoma which itself occurs in 0.1- 0.2 % of appendicectomies. The median age of presentation of appendicial adenocarcinoma is in the sixth or seventh decade of life and LAMN usually presents at more than 50 years of age. Patients commonly present with abdominal pain, though asymptomatic cases with incidental discovery of the lesion amount to one fourth of cases.1,2 We present here a case which presented as an obstructed inguinal hernia associated with a mucocele of appendix diagnosed as Low grade Appendicial Mucinous neoplasm post operatively.

Case Report

Clinical findings: A 45-year-old man presented with a two day history of acute onset pain and tenderness in right inguino-scrotal swelling and got admitted through surgical emergency. The swelling was present for last two years without any medical advice. Examinations revealed an afebrile patient with a huge tender swelling in the inguinoscrotal region. Overlying skin was healthy looking. Taxis was tried gently but failed . Peristaltic sound was present over the swelling. It was provisionally diagnosed by the surgeons as obstructed irreducible inguinal hernia with small bowel content. As the patient presented as an emergency case of obstructed hernia, and time was crucial radiological imaging could not be availed.

C-193 area. A densely adherent hernial sac was identified separately(Fig.1). Appendectomy was done and specimen was sent for histopathology. Peritoneal cavity did not show any fluid collection. Content of the hernial sac was noticed to be viable small bowel. Caecum and small bowel were reduced after confirming their viability and mesh hernioplasty was done. Mesenteric lymph nodes were unremarkable. Ileostomy, colostomy and orchidectomy could be avoided. Post operative recovery was uneventful except for slight induration and pain over the scrotum during follow up. Pathologic features: On gross histopathological examination, appendix was found to be hugely distended, measuring 5 cm X 2.5 cm; which on cutting open yielded mucoid material. No lymph nodes were sent with the specimen. On microscopy, the appendicial mucosa showed serrated and undulating morphology and it rested on the fibrous tissue. (Fig.2a, Fig.2b) Some areas showed the denudation of mucosa. Cytologically, the mucosal cells had low grade dysplasia.(Fig.2c) There was invasion of the mucoid material into the muscular layer.(Fig.3). The diagnosis was given as low grade appendicial mucinous neoplasm.

Discussion

Operative notes: After taking ileostomy and orchidectomy consent and performing the baseline investigations and serology, exploration was done with an inguino scrotal incision. It revealed a hugely distended viable caecum and appendix with mucoid material in surrounding

LAMN is a rare tumor of the appendix which was termed variously earlier as mucinous cystadenoma, or mucocele when it is cystically dilated. LAMN was coined in 4th edition of WHO classification to omit â&#x20AC;&#x153; adenomaâ&#x20AC;? as this lesion has potential of producing widespread disease.2 It is more common in females, usually observed in patients over 50 years age.3The most common presenting symptom associated with LAMN is abdominal pain. However, substantial numbers of patients are asymptomatic.

Fig. 1: Photograph showing intraoperative specimen of the appendix, separate from the hernia mass and showing distension.

Fig. 2a: photomicrograph showing morphology of the appendicial mucosa showing serrations and undulations (H&E, 100x)

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Lamn with Obstructed Hernia

Fig. 2b: photomicrograph showing mucosa resting on fibrous tissue (H&E ,100x)

directly

Fig. 2c: photomicrograph showing appendicial mucosa with low grade dysplasia, (H&E, 400x)

are small and regular with low grade dysplasia. Often there is atrophy and fibrosis of underlying submucosa and muscularis propria, but desmoplasia is absent. They tend to produce atrophy of the underlying lymphoid tissue.1 The morbidity/mortality associated with LAMN results from rupture and intraperitoneal spread of mucin-producing epithelium, which may cause pseudomyxoma peritonei, which warrants careful intraoperative tissue handling.7 LAMN is found to be associated with colonic and ovarian malignancy.7-9

Fig. 3: photomicrograph showing invasion of muscle layer by mucin (H&E, 400X)

Presentation as obstructed inguinal hernia along with separate LAMN is a very rare finding, and to our best knowledge, yet to be reported. However, presentations with bloody stool, intussusception have been reported.4 Imaging studies like ultrasonography, computed tomography are required for differential diagnoses as symptoms are non specific. The imaging studies also help to uncover incidental cases. However, the diagnosis is usually made intraoperatively or postoperatively on histopathological examination. 3,5,6 LAMN generally grow slowly, and tend to produce the clinical picture of low-grade pseudomyxoma peritonei in which spread beyond the peritoneum or nodal metastasis is unusual. Histologically, LAMN may have villous, serrated or undulating morphology, but unlike adenomas, they rest on fibrous tissue rather than lamina propria. Nuclei

Since there are no evidences in regard to lymphatic or hematogenous spreading of LAMN, if the mass confines in the appendix body without local invasion or caecal involvement, simple appendectomy and mesoappendix excision is considered sufficient treatment. For mass involving the caecum or adjacent organs, right hemicolectomy is often required.7-9

Conclusion

Low grade appendicial mucinous neoplasm, though a low grade neoplastic entity, can result in fatalities owing to development of pseudomyxoma peritonei. When the lesion is confined to the appendix only, simple appendectomy suffices. So, the aim of the diagnosis should focus at proper management and avoid confusions regarding nomenclature.

Acknowledgements

The authors are indebted to the technical staff of the Histopathology department and others in the Department of surgery and pathology, IPGME&R, Kolkata.

Declaration Funding none

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Competing interests none declared

Reference

1. Carr NJ, Sobin LH. Adenocarcinoma of the appendix In: Bosman FT, Carneiro F, Hruban RH, Theise ND,editors. WHO classification of Tumors of the Digestive System,4th ed Lyon, France: IARC; 2010. 122-125 2. Wang KC, Chen CH, Chen CZ, Huang HY, Jau JY, Lin TC et al. Low- grade appendicial mucinous neoplasm: a rare cause of acute abdomen. J Soc Colon Rectal Surgeon. 2012 3. Emmi S, Galasso MG, Ursino V, Scala R, Guastella T. Appendiceal mucoceles: a case report. Minerva Chir 1998; 53:807-10 4. Higa E, Rosai J, Pizzimbono CA, Wise L. Mucosal hyperplasia, mucinous cystadenoma, and mucinous

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cystadenocarcinoma of the appendix. A re-evaluation of appendiceal â&#x20AC;&#x153;mucoceleâ&#x20AC;?. Cancer 1973;32:1525-41. 5. Kim SH, Lim HK, Lee WJ, Lim JH, Byun JY. Mucocele of the appendix: ultrasonographic and CT findings. Abdom Imaging 1998;23:292-6. 6.

Madwed D, Mindelzun R, Jeffrey RB Jr. Mucocele of the appendix: imaging findings. AJR Am J Roentgenol 1992; 159:69-72..

7. Kahn M, Friedman IH. Mucocele of the appendix: diagnosis and surgical management. Dis Colon Rectum 1979;22:267-9. 8. Shayani V. Mucinous cystadenoma of the cecum missed at laparoscopic appendectomy. Surg Endosc 1999;13:1236-7. 9. Machado NO, Chopra P, Pande G. Appendiceal tumour retrospective clinicopathological analysis. Trop Gastroenterol 2004;25:36-9.

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Case Report Solitary Eosinophilic Granuloma of the Radius: A Case Report Junu Devi*1, Sukanya Kalita2 1

Department of Pathology, Gauhati Medical College, Guwahati, Assam. India 2 Department of Pathology, Wintrobe Hospital, Guwahati. Assam. India Keywords: Eosinophilic granuloma, Bone tumor, IHC

ABSTRACT Eosinophilic granuloma (EG) is an uncommon tumor of bone and soft tissue usually presenting in children. The clinical and radiological features of EG are highly valuable but not sufficient for diagnosis. Histopathology and immunohistochemistry give confirmatory diagnosis. We present here a case of EG localized to the radial head which was diagnosed histopathologically. Immunohistochemistry was done for CD1a, which was positive in our case confirming the diagnosis.

*Corresponding author: Dr Junu Devi, C-3 Kanika Apartment. Noonmati Lastgate,Guwahati.Assam India-781020 Phone: +91- 9435144568 E-mail: drjdevipath@gmail.com

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Introduction

Eosinophilic granuloma (EG) is a form of Langerhan cell histiocytosis (LCH) and was first described by Lichtenstein and Jaffer in 1940.[1] In 1953 Lichtenstein used the term Histiocytosis X for a group of diseases that included EG, Hand-Schuller-Christian disease and Letterer-Siwe disease. [1] All of them share common histogenesis and have similar morophological findings. It is considered a neoplasm of the mononuclear phagocytic system of unknown etiology. The disease is characterized by clonal proliferation of a special kind of histiocyte of the antigen- presenting dendritic type called the Langerhan cell (LC).[2] LCH predominantly affect children and young adults, but it can be found in any age group. The estimated incidence is about 5 per million, with most cases occurring in childhood.[3] Solitary EG accounts for the majority of the LCH cases usually involving bone and less commonly the lymph node, skin and lung.[4] The pathogenesis of LCH is ill understood, whether it is a reactive process or neoplastic is still not defined. Spontaneous resolution occurs is 1020% of patients and hence initial period of observation is often advisable. LCH is the preferred terminology over histiocytosis X. Among all three entities EG has got good prognosis; it may regress spontaneously and is extremely radiosensitive.

Immuno-histochemically the membranes of the Langerhan cells were positive for CD1a.(Fig2).She was treated by curettage and doing well on follow up.

Fig. 1: Microscopic picture of Eosinophilic granuloma (H &E ;X400)

Case Report

A six year old girl presented with complaints of pain and swelling over the right forearms for one and half months. There was no history of trauma, systemic examination was unremarkable, local examination revealed an ill defined swelling 3X1cm in diameter in the distal part of right forearm which was firm immobile and slightly tender. The overlying skin appeared normal. Routine hematological and biochemical investigations were within normal limit, X-ray radius showed a well demarcated 3x1cm oval osteolytic lesion in the head of the radius. In addition there was no soft tissue involvement outside the bone. Although clinical findings suggested diagnosis of tubercular osteomyelitis radiological findings did not favour such diagnosis. FNAC was done but smears were inadequate - scanty material showing mostly inflammatory cells predominantlty neutophils and eosinophils along with occassional histiocytic cells in a haemorrhagic background therefore repeat FNAC was advised. However biopsy material (curratage) was sent by the surgeon. Histopathological examination of the curetted material revealed proliferation of atypical Langerhan cells mixed with eosinophils and multinucleated histiocytic giant cells which was compatible with EG (Fig1). www.pacificejournals.com/apalm

Fig. 2: Immunohistochemical stain: Positive for CD1a

Discussion

EG is a rare bone tumor representing less than 1% of all bone tumors.[5] It is the mildest and commonest form of LHC.[6] It commonly affects children and young adults, particularly males. Any bone can be involved; the more common sites are the skull, mandible, spine, ribs and the long bones. [7] It can be asymptomatic or can present as localized pain, tender swelling and fever. Patient with EG fall into two groups - disease limited to either bone or soft tissue or combination of both. LCH can have a benign course or sometimes can present with a very diffuse disease.[8] LCH can be subdivided in to three clinico-pathological entities acute disseminated LCH (Letterer- Siwe disease) unifocal and multifocal unisystem LCH (EG), multisystem LCH (Hand Schuller Christian disease). It has a highly variable course with partial or complete healing of the lesion, recurrence after treatment, progression or spontaneous remission without treatment.[9] eISSN: 2349-6983; pISSN: 2394-6466

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Solitary Eosinophilic Granuloma

The radiological depiction of LCH (EG) is necessary as to determine the activity and nature of the tumor, plain X-ray depicts the size and borders of the lesion. CT and MRI demonstrate the exact size and borders of the tumor as well as the situation of the surrounding tissues and a probable hematoma. The clinical and radiological findings are often not specific enough to determine diagnosis.[10] Cytology is very helpful in diagnosis of EG of bone.[4] The classic cytological features of EG include high cellularity composed of sheets and scattered Langerhan cells with characteristic grooved, folded, indented nuclei admixed with polymorphous population of numerous eosinophils, neutrophils, lymphocytes, plasma cells, multinucleated giant cells and macrophages.[4,11] Charcot Leyden crystal may also be observed in the background.[12] Diagnosis is confirmed by histopathological examination and immunohistochemistry. Histopathologically EG is characterized by clonal proliferation of Langerhan type histiocytes, which are diagnostic.[13,14] These contain Birbeck granules (electron microscopic feature) whose role is yet unknown. Eosinophils, lymphocytes, fibroblasts, foam cells are not pathognomonic. Morphologically the key feature is the identification of Langerhans cells with characteristic grooved, folded or indented nuclei in the appropriate melieu that includes variable numbers of eosinophils & histiocytes including multinucleated forms.[4,11] An ultra-structural hallmark of LCH is the characteristics tennis racket-shaped Birbeck granules in the cytoplasm. [6] Immunohistochemically LCH is positive for CD1a and s100 protein, Langerin (CD207),MCH classII, PNA (peanut agglutinin).[18] CD1a is diagnostic. Important differential diagnoses are osteomyelitis, lymphoma, chondroblastoma, Ewings sarcoma, metastatic carcinoma.[4]A possibility of osteomyelitis, especially of tuberculous nature, is the most important differential diagnosis particularly in our country where tuberculosis is so common. But eosinophilic infiltrate and characteristic nuclear grooves are important differentiating points. In case of lymphoma, presence of eosinophils and histiocytes can mimic Hodgkins lymphoma but identification of ReedSternberg ( RS) cell should be mandatory for diagnosis of the disease. Again characteristic nuclear features of LH cells are absent in lymphoid cells. Immunohistochemical markers can also be helpful in differentiating the diseases.[4] Another differential diagnosis chondroblastoma also show nuclear groove and indentation and thereby resemble LC. However, LCH has a polymorphous population of cells including neutrophils, eosinophils and foamy histiocytes, rather than bimorphic population of chondroblasts and osteoclastic giant cells typical of chondroblastoma. LCH

also present with less numerous osteoclast-like giant cells.[15] In Ewing sarcoma characteristic small round cell morphology makes it easily distinguishable from LCH. Metastatic carcinoma especially renal cell carcinoma overwhelmingly exhibit a clustered cell morphology and epithelial features.[4] The etiology of LCH is unknown and there is continuing debate whether this condition is neoplastic or nonneoplastic. Studies of the x-linked androngen receptor gene have demonstrated a monoclonal proliferation of Langerhans cells in all major clinical syndromes.[6] Senechal et al[16] proposed that enhanced cell survival rather than uncontrolled LC proliferation as in neoplasia, is likely to play a major role in the maintenance and dissemination of these slow-growing tumors. The clinical course is related to the number of organs affected at presentation, with an overall survival of 95% for patients with unifocal disease;10% of patients with unifocal disease eventually progress to multisystem disease.[6]With multifocal disease,60% have a chronic course,30%achieve remission, and mortality is up to 10%.[17] Management of LCH (EG) is controversial because of unpredictability of outcome and possibility of spontaneous healing. Modalities vary from observation, curettage intralesional steroid, low dose radiation, high dose systemic corticosteroid and chemotherapy. Low dose radiation in 4-6 fractions may be used when the disease is extensive, inaccessible or if it threatens an important structure.[8] Solitary bone lesion (EG) may be amenable to excision or limited radiation, however systemic disease often requires chemo-therapy, use of systemic steroid is common, singly or as an adjunct to chemotherapy.

Conclusion

EG is an uncommon entity which poses difficulty in its diagnosis due to its variable clinical presentations. Biopsy and immunohistochemistry give confirmatory diagnosis.

Ackowledgement None

Funding None

Competing Interests None declared

References

1. Char DH, Ablin A, Beck stead J. Histiocytic disorder of orbit. An Opthalmol 1984; 16:867-73 2. Jon A, vinay K. White cells, lymph nodes, spleen and thymus. In: Ramzi SC, Vinay K, Tucker C eds.

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Robbins pathologic Basis of Disease 6th edn W.B. saunders Co. 1999:685.

10. Kitsoulis PV, Paraskevas G, Vrettakos A, Marine A. A case of Eosinophilic granuloma of the skull in an adult man: a case report. Cases journal 2009;2:9144

11. Mukhopadhyay S, Mitra PK, Ghosh S. Touton like giant cell in lymph node in a case of langerhan cell histiocytosis. Journal of cytol 2007; 24: 191-2. 12. Kemer PV, Mousavi A, Karimi M, Bedayat GR. Fine needle aspiration of Langerhan cell histiocytosis of the lymph node. A report of six cases. Acta cytol 2002;46:753-6 13. Lichtenstein L. Histiocytosis X eosinophilic granuloma of bone, lettere-siwe disease and schullerchristian disease).Further observation of pathological and clinical importance. J Bone joint surg Am. 1964; 46:76-90. 14. Sai S, Fujii K, Masui F, Kida Y. Solitary eosinophilic granuloma of the sternum. J orthop sci 2005; 10: 108111. 15. Fanning CV, Sneige NS, Carrasco CH, Ayala AG, Murrary JA, Raymond AK. Fine needle aspiration cytology of chondroblastoma of bone.Cancer 1990;65:1847-63 16. Senechal B, Elain G, Jeziorski E, Grondin V, PateyMariaud de serre N, Jaubert F, et al. Expansion of regulatory T- cells in patients with Langerhan cell histiocytosis. PLoS Med 2007;4:e253 17. Komp D, EI Mahdi A, Starling K, Easley J, Vietti T, Berry D, et al. Quality of survival in histiocytosis X: A Southwest Oncology Group study.med Pediatr Oncol 1980;8:35-40. 18. Kumar N, Sayed S, Vinayak S. Diagnosis of langerhan cell histiocytosis on fine needle aspiration cytology. A case report and review of the cytology literature. Pathology Res Int 2011;2011;439518.

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3. Nicholson HS, Egeler RM, Nesbit ME. The epidemiology of Langerhen cell histiocytosis. Hematol oncol clin North Am 1998; 12: 379-84. 4. Jain A, Alam K, Maheshwari V, Jain V, Khan R. solitary Eosinophilic granuloma of the Ulna: Diagnosis on fine needle aspiration cytology. Journal of Cytol 2008; 25:153-6. 5. Ando A, Hatori M, Hosaka M, hagiwara Y, Kita A, Itoi E. eosinophilic granuloma arising from the pelvis in children: A report of three case, Upsala J med Sci 2008: 113: 209-16. 6. Weiss LM, Grogan TM, Muller hermelink stein H, Dura T, Favara B, paulli M, Fller AC. Langerhan cell histocytosis. In: Jaffe ES, Harris NL, stein H, Vardiman JW, editors, pathology and genetics of tumor of haematopoietic and lymphoid tissue. Lyon: IARC Press; 2001; P: 280-2. 7. Kaul R, Gupta N, Gupta S, Gupta M. Eosinophilic granuloma of skull bone. J Cytol 2009; 26: 156-7. 8. Kramer TR, Noecker RJ, Miller JM, Clark LC. Langerhan cell histiocytosis with orbital involvement. Am J opthalmol 1997; 124:814-24 9. Chacha PB, Khong BT. Eosinophilic granuloma of bone. A diagnostic problem. Clin orthop 1971; 80:79-88.

Case Report Splenic Lymphoma with Villous Lymphocytes: A Case Report with Review of Literature Ruquiya Afrose*, Mohammed Akram, Nirupama P Khan, Usha Rusia Department of Pathology, J.N. Medical College, AMU, Aligarh, India Keywords: Splenic Lymphoma, Villous Lymphocytes, Leukemia

ABSTRACT Splenic lymphoma with villous lymphocytes (SLVL) is a rare low-grade B cell non-hodgkinâ&#x20AC;&#x2122;s lymphoma with distinct clinical and morphologic features. We report a case in a 55 year old man with the diagnosis of Chronic lymphocytic leukemia. On examination patient had no peripheral lymphadenopathy, but had splenomegaly of 2cm below costal margin. Laboratory investigations revealed WBC count 27.9x109/l with an absolute lymphocyte count of 22.3x109/l. Peripheral smear examination showed 80% lymphoid cells, 36% of which showed villous morphology, R-O nucleus, opened up chromatin and no nucleolus. Bone marrow aspirate and biopsy also showed involvement by these villous lymphocytes. These cells were negative for all cytochemical stains including tartrate-resistant acid phosphatase. On flow cytometric immuno-phenotyping these lymphoid cells were highly positive for CD19, CD20, FMC-7, moderately positive for CD10 and negative for CD5, CD23, CD25, CD103 and surface immunoglobulins (SIg) . Based on these findings a diagnosis of SLVL was suggested

*Corresponding author: Dr. Ruquiya Afrose, Senior Resident, Department of Pathology, J.N. Medical College, AMU, Aligarh, U.P. 202002, India Phone: +91-09219716166 E-mail: ruqs.afrose@gmail.com

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Afrose et al.

Introduction

Splenic Lymphoma with Villous Lymphocytes (SLVL) is a low-grade B cell non-Hodgkinâ&#x20AC;&#x2122;s lymphoma with distinct clinical and morphologic features that include splenomegaly without lymphadenopathy, lymphocytosis rarely exceeding 100x 109/L, and a discrete monoclonal band in the serum in one third of patients [1-3]. According to WHO 2008, it is considered as the leukemic counterpart of Splenic marginal zone lymphoma (SMZL) that comprises less than 2% of lymphoid neoplasm [4,5]. Because of the considerable overlap of clinical and morphological features between various chronic lymphoproliferative disorders (CLPDs) it requires immuno-phenotyping to distinguish from others and therefore is under diagnosed, as most centers in India lack this facility. As the treatment modalities and prognosis differ from those of the more common lymphoproliferative disorders, it is imperative to diagnose this condition for its proper management. We report a case in a 55 year old man who presented in the outpatient department with the diagnosis of Chronic lymphocytic leukemia (CLL).

Case Report

A 55 year married male, farmer by occupation, resident of Bihar was referred to GTB hospital in August 2011 as a case of CLL. Patient had complaint of self healing recurrent pruritic skin lesions especially on foot and palm and abdominal discomfort for last two years. Patient also had significant weight loss of 10kg in past 6 months. On examination patient had no peripheral lymphadenopathy, had splenomegaly 2cm below costal margin. Laboratory investigations revealed hemoglobin of 12.1gm%, WBC count 27.9x109/l with an absolute lymphocyte count of

Fig. 1: Peripheral smear showing villous lumphocytes

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C-201 22.3x109/l. Peripheral smear examination showed 80% lymphoid cells identified, with R-O nucleus, opened up chromatin, no nucleolus and moderate amount of cytoplasm, many of the cells show (36%) cytoplasmic projections which were broad based and circumferential and in some projections were confined to the poles (fig 1). These cells were negative for all cytochemical stains including tartrate-resistant acid phosphatase. Bone marrow aspirate and biopsy showed atypical lymphoid cells interspersed in between normal hematopoeitic cells with similar villous morphology as described in peripheral smear along with large no of bare nuclei (fig 2). Protein electrophoresis demonstrate polyclonal band in â&#x2013;Ą region. Flow cytometric immunophenotyping was performed on peripheral blood and the gated lymphocytes were highly positive for CD19, CD20, FMC-7, moderately positive for CD10 and negative for CD5, CD23, CD25, CD103 and SIg. Based on clinical presentation, morphology, bone marrow involvement & flow cytometry, a diagnosis of SLVL was suggested.

Discussion

SLVL, a low grade B-cell lymphoma described in detail by Melo et al [1]. According to WHO classification system, marginal zone B-cell lymphomas are classified into three clinically distinct subtypes: nodal marginal zone lymphomas, extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphomas), and SMZL. Now it is considered to be the variant of SMZL[4,5].

Fig. 2: Bone marrow aspirate and biopsy showing infiltration by villous lymphocytes

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Splenic Lymphoma with Villous Lymphocytes

It is a rare neoplasm and in western literature few case series has been reported and comprised less than 2% of all lymphoid neoplasm [6] however from India only few anecdotal case reports have been published [7]. Most patients of SLVL are over 50 years and there is an equal sex incidence. SLVL is considered to be of B-cell origin of unknown differentiation stage, however presence of Ig gene somatic hypermutation in 50% of cases suggests exposure to antigen in the germinal centre microenvironment.

types of cells may not be immediately apparent by light microscopic examination alone, and electron microscopic studies, or cell volume measurements, may be needed [1]. In the present case we observed villous lymphocytes in 36% of total peripheral lymphocytes and in 25% of bone marrow aspirate. These lymphocytes had round to oval nucleus, opened up chromatin, no nucleolus and moderate amount of cytoplasm showing cytoplasmic projections which were broad based and in some of the cells these projections were confined to the poles. The bone marrow showed a characteristic nodular interstitial infiltration with no reticulin fibrosis unlike the fine reticulin fibrosis seen in HCL [10]. In addition to morphological features, SLVL differs from HCL as it lacks tartrate resistant acid phosphatase staining which is a hallmark of HCL [7]. Moreover, in one third of cases, peripheral blood may show a monoclonal band in serum protein electrophoresis [3] . Present case was negative for TRAP and no monoclonal band in protein electrophoresis could be demonstrated. Immunophenotypically, SLVL cells have variable profile of antigen expression. No surface marker in isolation is characteristic of SLVL, and therefore a complete panel of markers is required to differentiate it from other chronic lymhoproliferative disorders (table 2).

Although SLVL is specific disease, the villous lymphocytes are biologically related to neoplastic cells of other types of chronic lymphoproliferative disorders (table 1). Of these, CLL and PLL can be distinguished on the basis of clinical presentation, cell morphology and characteristic immunophenotypic profile. However others such as HCL and HCL-V and SLVL pose some difficulty as all three present with villous lymphocytes in peripheral blood [1,8]. Morphology of villous lymphocytes shows a distinct pattern of villous distribution as well as the nuclear characteristics and nucleoli which may sometimes help in distinguishing these three entities. Thus villous lymphocytes of hairy cells and HCL-V are typically circumferential and homogenously distributed whereas those of SLVL characteristically have preferential polar distribution of short and thin villi or are sometimes long and broad based. Beside this HCL and HCL-V have centrally located nucleus with abundant cytoplasm. HCL-V also shows a distinct nucleolus not seen in SLVL [1, 9] . In some cases the distinction between the three

In the present case, mature B-cell markers (CD19, and CD20) were highly positive however the absence of CD5& CD23 ruled out the diagnosis of CLL [1, 8]. There was high positivity for FMC-7 in our case. As FMC-7 is also highly positive in HCL and HCL-V, the negative expression of CD25 and CD103 were helpful in ruling out HCL [1, 6].

Table1: Clinical and haematological presentation of chronic lympho-proliferative neoplasms. Parameters Clinical Presentation Age (Yr) Organomegaly

CLL Asymtomatic

>65 Moderate to Massive Spleen& liver, Lymphadenopathy Present Course& Prognosis >10yrs, indolent

PLL B symptoms +

SMZL/SLVL Insidious

HCL B symptoms

HCL-V B symptoms

>60 Massive spleen,

>50 Spleen

40-70 Massive spleen

40-70 Moderate spleen

minimal Poor (3-5 yrs)

nil Indolent, poor response to therapy B cell of U/K Differentiation stage <1 Lakh

nil Nil >10yrs, responsive to No response to IFN-Îą IFN-Îą & Cladirabine & purine analogues

Type Of Cell

Mature Ag prolymphocytes expressing B cell

TLC

>100x109/L

Bone Marrow Involvement

diffuse

Intrasinusoidal, Interstitial, nodular

Late activated memory B cell

activated memory B cell, late stage

<10,000 (usually pancytopenia) Diffuse, fried egg appearance, reticulin fibrosis

>35,000 Nodal interstitial, No significant fibrosis

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Table 2: Comparison of immunophenotypic features in various chronic lymphoproliferative disorders CD5 CLL PLL FL SLVL HCL

++ -/+ -

CD19, CD20 ++ ++ ++ ++ ++

HCL-V

CD23

CD10

CD25

C103

CD123

Annexin-A1

FMC-7

SmIg

++ -/+ -/+ -

-/+ + -/+ -

-/+ ++

-/+ ++ ++ -/+ ++

-/+

Weak Strong Strong Strong Strong to moderate Weak to strong

+ expressed, ++strongly expressed, - not expressed, -/+may be expressed, Immunophenotypic profile of SLVL and HCL-V shows considerable overlap, CD11c is one marker which shows positivity in HCL-V as opposed to SLVL where it is variable. However the diagnosis cannot be solely confirmed based on immunophenotype alone and therefore taking a holistic view based on the clinical presentation, morphology, bone marrow involvement and immunophenotypic profile a diagnosis of SLVL was considered over HCL-V.

3. Berger F, Felman P, Thieblemont C, Pradier T, Baseggio l, Bryon PA et al. Non-malt marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients. Blood 2000;95:1950-6.

Conclusion

5. Bociek RG. Splenic marginal zone lymphoma: villous, not necessarily villainous. Oncology (Williston Park). 2012;26:208-10.

The clinical course of SLVL is indolent, even with bone marrow involvement. These patients are usually non responsive to usual chemotherapy regimens, typically effective in other chronic lymphoid leukaemias. These patients typically have haematologic responses to splenectomy with long term survival, hence it is important to diagnose it as a separate entity.

Acknowledgements

Dr Sarla Aggarwal, Ex Professor and Head of Dept of pathology, UCMS & GTB Hospital New Delhi

Funding: None

Competing interest None declared

Reference

1. Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D. Splenic B cell lymphoma with circulating villous lymphocytes: Differential diagnosios of B cell leukaemias with large spleens. J clin pathol 1987; 40: 642-51. 2. Mulligan S, Matutes E, Dearden C, Catovsky D. Splenic lymphoma with villous lymphocytes: natural history and response to therapy in 50 cases. Br J Haematol 1991;78:206-9.

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4. Swerdlow SH, Campo E, Harris NL, et al (Editors). WHO classification of tumours of hematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer Press; 2008.

6. Armitage JO, Weisenburger DD. New approach to classifying non-hodgkin’s lymphomas: clinical features of the major histologic subtypes. Nonhodgkin’s lymphoma classification project. J clin oncol 1998;16:2780-95. 7. Gupta R, Naseem S, Sukumaran S, Kashyap R, Kaur S, Paul L. Splenic lymphoma with villous lymphocytes. Indian J Pathol Microbiol 2008;51: 113-5. 8. Matutes E, Morilla R, Owusu-ankomah K, Houlihan A, Catovsky D. The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B cell disorders. Blood 1994;83:1558-62. 9. Bethel KJ, Sharpe RW. Pathology of hairycell leukaemia. Best Pract Res Clin Haematol. 2003;16:15-31. 10. Cawley JC. The pathophysiology of the hairy cell. Hematol Oncol Clin North Am 2006;20:1011-21. 11. Thieblemont C, Felman P, Callet-Bauchu E, TraverseGlehen A, Salles G, Berger F et al. Splenic marginal zone lymphoma: a distinct clinical and pathological entity. Lancet oncol 2003;4:95-103.

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Case Report Soft Tissue Chondromas: A case series of 6 cases with review Ankit Kaushik*, Amita Malik, Rajni, S Batra, V K Sharma, Geetika Khanna Department of Pathology, VMMC and Safdarjung Hospital Delhi, India Keywords: Soft Tissue Chondroma, Cytology, Cartilagenous tumor, Soft Tissue Tumor

ABSTRACT Soft tissue chondroma is extraskeletal benign cartilagenous soft tissue tumor rare to their skeletal counterpart enchondromas. They manifest characteristic radiological and histological features with consistent cytological features. Although several histologic studies of soft tissue chondromas have been published, correlative studies of cytologic, radiologic and corresponding histologic features are limited. To better define the triad of cytological, radiological and histological features of various Soft tissue chondroma, we reviewed the radiological and corresponding cytohistologic material of six tumors. The radiological finding consists of a well defined soft tissue mass, partly calcified not attached to underlying bone. Classical cytological patterns were found to be small clusters of benign appearing chondrocytes along with chondromyxoid fragment, with a impression of soft tissue chondroma. Post operatively all six cases show characteristic histologic features consisting of adult type hyaline cartilage with mild atypia in chondocytes in one and inflammation in other tumor. The cyto-histologic features along with radiologic feature constitute definitive triad in diagnosing soft tissue chondromas.

*Corresponding author: Ankit Kaushik, B 19 Mahesh Park Modinagar Ghaziabad UP, India Phone: +91-9953816240 E-mail: kaushikankit30@yahoo.co.in

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Kaushik et al.

Introduction

Soft tissue chondromas (STC) are rare benign soft tissue tumors occurring in extraosseous and extra-synovial location predominantly composed of adult type hyaline cartilage1. Because of their rarity they are liable to be under diagnosed clinically2. The STC share same pathology as there bony counterpart except there lacations3,4. The current WHO classification places STC under category of chondro– osseous tumors under benign tumors. The majority of patients are middle aged persons with age range of 30-60 years with specific predilection for hands and feet1,5. The STC are slow growing tumors with special predilection to extremities especially acral areas like fingers1. Our study entailed a retrospective review of the clinicopathological features of six histologically confirmed cases of STC, received in Central Institute of Orthopedics, laboratory Safdarjung Hospital over past four years.

Case Reports

We report our ongoing experience involving six cases of STC with three FNAC soft tissue aspirates reported in CIO Laboratory over four years. All the relevant history was retrieved from the requisition form with available radiological, cytological and histological data. The X ray findings available in all the six patients were noted, all of which suggest a possible radiological diagnosis of soft tissue chondroma. The cytologic aspirate was done in three patients without general anesthesia using 23 gauge needles by the standard manual method at CIO laboratory VMMC and Safdarjung Hospital Delhi. The available gross findings were noted. Histologic material in the form of paraffin wax blocks and H&E stained slides were retreived

C-205 for all six cases. All six cases were reported by total of two experienced pathologists specialized in reporting of musculoskeletal lesions over more than 10 years. The cases were again reviewed in light of available radiological and cytological diagnosis. In our study the age group of affected patients showed a wide range varying from 12 to 40 years with a mean age of 27.67 years. Males and females were found to be equally affected. Most patients presented with a solitary, painless lump of long duration arising in the vicinity of tendons and joints. All patients were found to have a hard, fixed, non-tender swelling with well defined borders involving extremities mainly distal extremities. A diagnosis of a benign chondroma arising from the soft tissues of the distal extremities was made based on the clinico-radiologic and cytologic findings. Plain X-ray was done in all patients and FNAC was undertaken in three out of six patients. Plain radiograph in all the six cases revealed a soft tissue mass, with normal underlying bone (Figure 1). No bony erosion or periosteal reaction was observed. Dense homogenous calcification was seen in two out of six cases. CT scan (Figure 2) done in one patient showed a soft tissue mass partly calcified, separated from the underlying bone. Extensive calcification masking the cartilaginous component of the tumor was noted in one of the cases. Cytological smears of three cases available out of six cases shows myxo-chondroid fragments (Figure 3) suggestive of a benign tumour of cartilaginous origin and a diagnosis “consistent with a STC” was made in view of available radiological finding.

Fig. 1: X-ray right ankle shows well circumscribed soft tissue mass, partly calcified, no underlying bony connection was appreciated.

Fig. 2: Low power view of aspirate showing chondromyxoid fragment in hemorrhagic background. (Giemsa; 100x)

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Soft Tissue Chondromas bone. Exact sub typing on cytology could be done in three of the cases. On cytology along with clinico-radiological findings, they were labeled “consistent with STC”. Microscopically soft tissue chondromas are composed of mature hyaline cartilage with chondrocytes cells identified in lacunae11. STC with predominant cartilaginous component constitute chondroblastic variant of soft tissue chondroma12. Various types of STC are identified with prominent fibrous, ossification or myxoid changes can be found designating tumors as osteochondromas13 or myxochondromas14

Fig. 3: Photo micrograph showing soft tissue mass with central foci showing lobule of cartilage with bony trabeculae (H&E 100x).

The complete excision of all swellings was done. The swellings varied in size from 1 to 5 cm in maximum dimension; were well circumscribed, lobulated and on cut surface exhibited a firm, gritty, grey and glistening appearance, with occasional myxoid and cystic areas. The tumour-bone interface was unremarkable. Histologicaly the lesions were composed of lobules of mature, adult hyaline cartilage; chondrocytic cells which were showing mild variation in size and shape were identified in the lacunae (Figure 4). One case showed moderate atypia but atypical mitotic figures were not found. Intralesional ossification was appreciated in three cases (Figure 5). Striking histiocytic reaction at the periphery of the lesion was noted in one case. No multinucleated giant cell or well formed granulomatous reaction was seen.

Discussion

This retrospective study was carried out in CIO laboratory over a period of four years. Our series included 6 cases of STC, with the youngest patient being 12 years of age and the oldest 40 years. The average affected age was 28.3 years. There were 3 males and 3 female. Chondroma of the soft tissue is usually asymptomatic with no reported sex predominance6 or a slight male predominance1,7. Foot was the site of involvement in five of our cases, as found by Ekanem and co-workers8 in their series and one case was in hand however finger. All the patients presented with soft tissue mass of long duration in acral areas from months to years. The radiological investigations showed STC to be well demarcated, lobulated with central calcification in some cases1,9,10 . The origin of these tumors are considered to be the tenosynovial sheath or the peritendinous soft tissue of the foot and failed to demonstrate any connection with

STC also present rarely with chondroblastic features15. The center of tumor lobules may have calcification masking normal cartilaginous component. Rare feature includes granuloma like reaction with epitheloid and multinucleated giant cells. Occasional cells may show mild nuclear atypia or mitosis but abnormal mitosis is never seen1. Thool and co-workers16 reported that the clinical, radiologic and cytologic triad is important for the correct cytologic diagnosis of soft tissue chondroma despite worrisome cellular atypia. STC can present from an immature pattern dominated by chondroblasts to a mature chondrocytes. The matrix can be hyaline, fibrous or fibrohyaline with granuloma-like areas in the stroma. The variable cellularity along with cellular immaturity and atypia could mislead one to a malignant pathological interpretation. However, a STC is unlikely to display cords of tumor cells within a myxoid matrix, as found in the extraskeletal myxoid chondrosarcoma or the primitive round-cell pattern. Furthermore, hemangiopericytoid vascularity is characteristic of the extraskeletal mesenchymal chondrosarcoma. Benign lesion showing cartilaginous component comes in differential diagnosis of STC. Calcifying aponeurotic fibroma can show foci of cartilaginous metaplasia in dense, poorly circumscribed fibromatous background. Tumoral calcinosis, giant cell tumor may come in differential diagnosis but shows characteristic histopathological feature. Synovial chondromatosis also shows similar histopathological feature like STC but it is present in large joints. The presence of atypia makes the diagnosis of STC difficult. Important differential diagnosis is well-differentiated extraskeletal chondrosarcoma, extraskeletal myxoid chondrosarcoma, and mesenchymal chondrosarcoma. While well-differentiated extraskeletal chondrosarcoma shows atypia, absence of abnormal mitoses and necrosis tilts diagnosis in favour of STC. Mesenchymal chondrosarcoma is another chondroid lesion which can

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Kaushik et al. show a well-differentiated cartilage surrounded by small, undifferentiated tumor cells. There are no specific clonal chromosomal abnormalities detected by STC. The pathogenesis remains unclear, involvement of chromosomes six and eleven has been implicated in some studies. Repeated micro trauma may be an initiating factor and was noticed in two of our patients. Follow up could be obtained in four cases, in which the results were found to be excellent with no report of recurrence at the end of two years. Local excision is curative although local recurrence is common.

Conclusion

STC are rare tumors leading to difficult clinical diagnosis. FNAC in conjunction with detailed clinical and radiographic data may allow a preoperative diagnosis of STC and permit planning of a therapeutic approach for them. STC can show worrisome histological atypia simulating chondrosarcoma, making it imperative that the clinical, radiological and histological triad is taken into account for arriving on the correct diagnosis.

Acknowledgements none

Funding None

Competing Interests None declared

Reference

C-207 5. Dahlin DC, Salvador AH. Cartilaginous tumors of the soft tissues of the hands and feet. Mayo Clin Proc 1974;49: 721-6. 6. Faraj A. A Soft tissue chondroma in a finger: a case report. Acta Orthopaedica Belgica, 2002;68:3. 7. Dahlin DC, Salvador AH. Cartilaginous tumors of the soft tissues of the hands and feet. Mayo Clin Proc 1974;49: 721-726. 8. Ekanem, Victor J, Adeoye II, Marchie TT. Recurrent extra skeletal soft tissue chondroma in a black african female: report of a case and review of literature. J Med Biomed Res.; 2006;5:18-21. 9. Bansal M, Goldman AB, DiCarlo EF, McCormack R. Soft tissue chondromas: diagnosis and differential diagnosis. Skeletal Radiol 1993;22: 309-15 10. Zlatkin MB, Lander PH, Begin LR, Hadjipavlou A. Soft-tissue chondromas. AJR Am J Roentgenol 1985;144: 1263-7. 11. Goyal P, Sehgal S, Ghosh S, Mittal D, Singh S. Extraskeletal Chondroma of Anterior Abdominal Wall in a Child. APSP Journal of Case Reports. 2013;4(3):54. 12. Legeai-Mallet L, Margaritte-Jeannin P, Lemdani M, Le Merrer M, Plauchu H, Maroteaux P, et al. An extension of the admixture test for the study of genetic heterogeneity in hereditary multiple exostoses. Hum Genet 1997; 99: 298-302

1. Nayler S, Heim s. Soft tissue chondroma. In: Fletcher CD, Unni KK, Mertens F (Eds). Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC Press; 2006. pp. 180-1. 2. Khedhaier A, Maalla R, Ennouri K, Regaya N. Soft tissues chondromas of the hand : A report of five cases. Acta Orthop. Belg. 2007:73;458-61. 3. Haktanır NT, Demir Y, Haktanır A, Aktepe F, Sancaktar N. Concurrent Soft Tissue Chondroma and Periosteal Chondroma of Thumb. Eur J Gen Med 2009; 6: 257-61. 4. Bell WC, Klein MJ, Pitt MJ, Siegal GP. Molecular pathology of chondroid neoplasms: Part 1, benign lesions. Skeletal Radiol 2006; 35: 805-13.

13. Reith JD, Bauer TW, Joyce MJ. Paraarticular osteochondroma of the knee: report of 2 cases and review of the literature. Clin Orthop 1997; 225-32.

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14. Weiss SW, Goldblum JR. Cartilagineous soft tissue tumors. In: Enzinger and Weiss’s Soft Tissue Tumours. 4th ed. Mosby-Harcourt: Philadelphia 2001; pp. 13611388. 15. Isayama T, Iwasaki H, Kikuchi M. Chondroblastomalike extraskeletal chondroma. Clin Orthop 1991;214-217. 16. Thool AA, Raut WK, Lele VR, Bobhate SK. Fine needle aspiration cytology of soft tissue chondroma. A case report. Acta Cytol. 2001;45(1):86-8.

Letter to Editor Cytopathology of Intramuscular Myxoma Shilpi Agarwal, Shivali Sehgal*, Preeti Rai, Priya Thomas Department of Pathology, Lady Hardinge Medical College, New Delhi, India Keywords: Myxoma, Cytology, Intramuscular

Sir,

Intramuscular myxoma (IMM) is a benign soft tissue tumor involving the musculoskeletal system (incidence of 0.1-0.13/100,000).[1] It usually occurs in large muscles of the thigh, shoulder, buttocks and arms. We report a case of an IMM diagnosed on fine needle aspiration cytology (FNAC). A 30 year female presented with an upper back swelling which was gradually increasing in size for the past 3 years. It was painless, slightly mobile and fluctuant. There was no history of prior trauma. Ultrasonography revealed a well defined, cystic lesion in the trapezius muscle (with no increase in vascularity on Doppler). A diagnosis of an intramuscular space occupying lesion was made. FNAC performed from multiple sites yielded 5ml of gelatinous aspirate; following which the swelling markedly decreased in size. Smears were hypocellular showing dispersed spindle and stellate shaped cells against a myxoid background. (Figure 1) Myxoid material can be seen in a variety of benign and malignant soft tissue lesions. Myxolipomas show presence of adipocytes while myxoid neural tumors have characteristic spindle cells with buckled nuclei. Myxoid liposarcoma contains lipoblasts and plexiform blood vessels; myxoid chondrosarcoma has presence of a chondroid matrix while low grade fibromyxoid sarcoma has cellular smears with spindle shaped cells. Myxofibrosarcomas have cells with nuclear atypia and curvilinear blood vessels. There were no adipocytes/lipoblasts/neural cells, no nuclear atypia/mitotic activity/necrosis and the presence of stellate cells against an abundant myxoid matrix suggested a diagnosis of myxoma. Excision was performed and we received a globular, cystic, encapsulated soft tissue mass (diameter=4.5cm). On cutting

open, it was unicystic and filled with mucoid material, wall thickness=0.2-0.4cm. No solid, fleshy or lipomatous area was identified. Sections showed an encapsulated lesion surrounded by skeletal muscle bundles; composed of spindle and stellate cells in a loose, myxoid matrix. Alcian blue staining (pH=2.5) was positive in the myxoid areas. There were no hypercellular areas, cellular atypia, mitosis or necrosis. Cells were CD34 positive and S-100 negative. The diagnosis was consistent with intramuscular myxoma. (Figure 2) IMM are common in the 5th and 6th decade with slight female predominance.[1] They may attain a large size and mimick sarcomas clinically. Myxoid material is a non specific, common finding in a large number of soft tissue tumors. Due to abundance of myxomatous tissue in IMM and poor cellularity, diagnosis on FNAC is difficult.[2] Silver et al found that only 3 out of 8 cases of IMM were correctly diagnosed on cytology.[3] Caraway et al studied cytology of 10 cases of IMM and found presence of spindle, stellate and histiocytoid cells in an abundant mucoid matrix.[4] Akerman et al analysed 10 cases of IMM and found good cyto-histological correlation.[5] IMM should be considered in the diagnosis of isolated firm to cystic lesions in the given clinical setting and be differentiated from other tumors. The present case highlights the cytological features of different myxomatous lesions. It is suggested that proper sampling of the lesion from multiple sites be performed to reach a definitive diagnosis. Although, FNAC can be used as a preliminary investigative tool, confirmation is obtained only on histopathology.

*Corresponding author: Dr Shivali Sehgal, Department of Pathology, Lady Hardinge Medical College, New Delhi, 110001, India Phone: +91-9818770874 E-mail: shivalisehgal@gmail.com,

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Cytology of Intramuscular Myxoma

Fig. 1: Hypocellular smear showing presence of stellate shaped cell. (Giemsa, 100X) Insets show cluster of plump cells (1), scattered spindle cell (2) and a stellate cell (3) from left to right.

Acknowledgements None

Funding None

Competing Interests None declared

Reference

1. Ozbek N, Danaci M, Okumus B, Gursel B, Cakir S, Dabak N et al. Recurrent intramuscular myxoma: review of the literature, diagnosis and treatment options. Turk J Cancer 2006;36:75. 2. Choukimath MS, Rangappa PK. Fine needle aspiration cytology of soft tissue tumors with special emphasis on grading of spindle cell sarcomas. International Journal of Applied Biology and Pharmaceutical technology

Fig. 2: Stellate cells dispersed in a loose, myxoid matrix. (H&E, 400X)

2012;3:47-60. 3. Silver WP, Harrelson JM, Scully SP. Intramuscular myxoma: a clinicopathologic study of 17 patients. Clin Orthop 2002;403:191-7. 4. Caraway NP, Staerkel GA, Fanning CV, Varma DG, Pollock RE. Diagnosing intramuscular myxoma by fine-needle aspiration: a multidisciplinary approach. Diagn Cytopathol 1994;11:255-61. 5. Ă&#x2026;kerman M, Rydholm A. Aspiration cytology of intramuscular myxoma: a comparative clinical, cytologic and histologic study of ten cases. Acta Cytol. 1983;27:505-10.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Letter to Editor Tumor or Gossypiboma Pankaj Gupta*, Shruti Chaudhary, Noopur Gupta, Sanjay Deb, Sumedha Kotwal, Ramesh Dawar Department of Pathology, Dharamshila Hospital and Research Centre, New Delhi, India

Dear Sir,

Surgical sponges are the most common type of retained foreign body.[1] The condition is called Gossypiboma. The term Gossypiboma, derived from the Latin word “Gossypium” (cotton) and the Swahilli “Boma” (place of concealment) denotes retained surgical sponge. [2] As this carries a medico legal implication, due care should be taken when diagnosing it and strict measures taken to prevent this complication from arising after surgery. Gossypiboma is most frequently diagnosed in the abdominal cavity and abdominal sponge is the most common foreign body reported. [3, 4] Its was first reported by Wilson et al in 1884 and since then it has been reported as 1 in 100 to 3000 for all surgical interventions and 1 in 1000 to 1500 of all intraabdominal operations. [4] A 45 year old diabetic female presented with complaints of abdominal distension and dull aching pain. She gave a history of cholecystectomy seven years ago at another hospital. On examination there was a scar of cholecystectomy and a central huge abdominal mass. The mass had ill defined borders with restricted mobility. Routine investigations were Hb-12.8 gm %, TLC- 7,700/ cumm, Platelets 1.84 lac/cumm. USG whole abdomen showed a large intra-abdominal mass without any clear organ of origin. Provisional diagnosis of ovarian mass was made. CA-125 levels were 57.20 U/ml (Normal <35 U/ ml). CT scan was done and showed benign mesentric cyst measuring 16.65x16.93x11.75 cms with enlarged uterus having multiple fibroids. Patient underwent exploratory laparotomy. Transverse meso colectomy, mesentric cyst excision with total abdominal hysterectomy and bilateral salpingooophorectomy was performed, and the specimen

was sent to the pathology department. On gross examination there was a cystic mass measuring 19.0 x 18.0 x 13.0 cms (Fig 1). Outer surface showed attached mesentery with adherent blood clots. On cutting open cyst was filled with brownish fluid and there was a piece of gauze with surgical tape measuring 5 cms long attached to inner surface of cysts wall covered with yellow exudate(Fig 2). Uterus and cervix received separately measured 11.5 x 8.0 x 5.5 cm, endometrium 3.0 cm with myometrium showed multiple intramural fibroids varying in diameter from 0.4 cm to 3.0 cm. Right and left ovary showed multiple cysts filled with serous fluid. Bilateral fallopian tubes were unremarkable. Microscopic examination revealed a cyst wall formed by fibrocollagenous tissue, numerous multinucleate foreign body type giant cells with intracytoplasmic refractile foreign bodies. No atypia seen. Sections from mesentery were unremarkable. Diagnosis of gossypiboma was made. Cervix showed features of chronic cervicitis with squamous metaplasia, endometrium was in proliferative phase with myometrium showing multiple intramural fibroids. Bilateral ovaries showed follicular cysts and bilateral fallopian tubes were unremarkable. Gossypiboma or a mass of cotton retained following surgical intervention is a rare clinical complication. The sponges are inert tissue and do not undergo decomposition. The clinical presentation of gossypiboma varies and depends on the location of the sponge and the type of reaction. Body can respond to the retained sponge in two ways. The first type is an exudative, acute inflammatory response with the formation of abscess in proximity to the retained sponge. This response usually leads to early detection and

*Corresponding author: Dr. Pankaj Gupta, Department of Pathology, Dharamshila Hospital and Research Centre, New Delhi-110096, India Phone: +91-9827624626 E-mail: drpankaj9@gmail.com

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Tumor or Gossypiboma

Fig. 1: Gross Specimen of Abdominal Lump

Fig. 2: Cut section showing Surgical tape and gauze piece

surgical removal. The second type is aseptic with fibrotic reaction to the cotton material and development of mass. This response usually presents with mass or abdominal pain or more commonly is an incidental finding. [3] This case is peculiar as it has presented seven years after surgery and had elevated levels of CA 125 thus mimicking an ovarian tumor.

Funding

Gossypibomas are uncommon, mostly asymptomatic and hard to diagnose. Particularly, chronic cases do not show specific clinical and radiological signs for differential diagnosis. It should be included in the differential diagnosis of soft-tissue masses detected in patients with a history of a prior operation. Although human errors cannot be completely avoided, continuous medical training and strict adherence to rules of the operation room should reduce the incidence of Gossypiboma to a minimum.

1. Aminian A. Gossypiboma: a case report. Cases Journal 2008;1:220

Acknowledgements None

None

Competing interests None declared

Reference

2. Ray S, Das K. Gossypiboma presented as abdominal lump seven years after open cholecystectomy. J. Surg. case rep. 2011:2. 3. Moyle H, Hines OJ, McFadden DW. Gossypiboma of the abdomen. Arch Surg. 1996; 131:566-8. 4. Kholi S, Singhal A, Tiwari B, Singhal S. Gossypiboma, Varied presentation: A Report of Two Cases. J Clin Imaging Sci.2013;3:11.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Letter to Editor An Umbilical Polyp in an Infant- A Rare Congenital Lesion Meghna Singh*1, Jitendra Chaudhary2 Department of Pathology, Shankar Diagnostic Centre, Agra, India Department of Radiodiagnosis, Shankar Diagnostic Centre, Agra, India 1

Dear Sir,

In early embryogenesis, the vitelline duct connects the alimentary canal and the yolk sac. By the time of birth, this communication channel is usually obliterated. However, remnants of the embryonic communication may persist as a lesion on the umbilicus that is called an omphalomesenteric (vitelline) duct remnant. The lesion should be recognized and treated early because of its potential to coexist with life-threatening anomalies. A 2 month-old boy was referred to the pediatric surgical unit for evaluation of a nonhealing umbilical remnant. Her mother reported persistent drainage and occasional bleeding from the site. The results of a physical examination revealed an otherwise healthy infant with a bright cherry-red, glistening, 1 cm nodule on the umbilicus (Figure1). Ultrasonographic evaluation of the lesion revealed a moderately vascular hypoechoic mass lesion (approx 10x8 mm) in the region of umbilicus (D/DCongenital vascular malformation/ Umbilical polyp) . Grossly, the lesion measured 1 x 0.6 cm. Consistency was firm and cut surface showed solid white areas. Results of a microscopic evaluation showed a tissue composed of small intestinal mucosa showing intestinal villi and crypts. Beneath it were bundles of smooth muscle fibres and fibrovascular stroma. Interglandular area showed ectatic vessels (Figure 2). The patient was referred for exploratory surgery. During laparotomy, no evidence was found of an associated Meckelâ&#x20AC;&#x2122;s diverticulum, an omphalomesenteric duct, or a patent urachus. The umbilical nodule was excised, and its base was cauterized. The patient recovered uneventfully. Early in human embryogenesis, the alimentary canal communicates with the yolk sac through the umbilicus. As the embryo grows, the communicating omphalomesenteric

duct narrows; by the fifth week of gestation, it is surrounded by the growing umbilical cord.[1] The duct normally loses any vestige of its former existence and disappears by about the sixth week.[1] Under abnormal conditions, part of the omphalomesenteric duct persists, resulting in anomalies. The most common may be Meckelâ&#x20AC;&#x2122;s diverticulum, which occurs in about 2% of the population.[2] This remnant is found 30 to 60 cm proximal to the adult ileocecal junction on the antimesenteric surface of the small bowel. It may be connected to the umbilicus through a fibrous tract. Other anomalies include a stand-alone fibrous tract, cysts within that tract or within the abdominal wall at the umbilicus, an umbilical sinus, or an external polyp at the umbilicus.[2] Cysts within the fibrous tract may migrate into the umbilical cord and cause fetal death.[3] Finally, the remnant may persist as an open umbilical enteric fistula or a patent vitellointestinal duct connecting the lumen of the small intestine to the external surface through the umbilicus.[4] The patent omphalomesenteric duct is the most problematic vitelline remnant. A confirmed patent omphalomesenteric duct, especially a short one, is considered a surgical emergency because of its high risk of prolapse and herniation. External remnants of the omphalomesenteric duct occur as a brightred polyp. The polyp may communicate with a patent duct or sinus. Mucosal secretions give it a sticky surface. When gastric like glandular elements are present, the polyp may be surrounded by severely erosive irritant contact dermatitis. For uncomplicated polyps, surgical excision may be adequate.[5] Pathologic diagnosis requires the presence of ectopic gastrointestinal epithelium. Steck and Helwig[1] reviewed 40 cases of omphalomesenteric remnant at the Armed Forces Institute of Pathology. Fourteen of these patients were adults. Thirteen of the 40 patients had gastric mucosa (predominant in 9); 25 patients had small bowel mucosa (4 mixed with gastric mucosa); 5 had predominantly large bowel mucosa; and 1 had ectopic pancreas.

*Corresponding author: Dr Meghna Singh, 10 Laxman Nagar, Near Arjun Nagar, Agra, Uttar Pradesh, 282001, India Phone: +91-8126738984 E-mail: dr.singhmeghna@gmail.com

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An Umbilical Polyp in an Infant

Fig. 1: An umbilical polyp seen as a bright, glistening, cherry red nodule on umbilicus

The differential diagnosis of umbilical polyp includes umbilical granuloma, which is darker red, more apt to bleed, and responsive to silver nitrate cauterization, and also vascular neoplasms, including sarcomas and congenital hemangiomas (Table 1). It also includes a patent urachus, whose presence is confirmed by the passage of urine through the umbilicus.[6] An inadvertently ligated loop of bowel that has herniated into the umbilical cord also may simulate a patent omphalomesenteric fistula, especially when there is an intestinal fistula.[6] TABLE 1: Differential Diagnosis of Vitelline Umbilical Remnant Differential Diagnosis of Vitelline Umbilical Remnant Vitelline remnant Umbilical (pyogenic) granulomas Vascular neoplasms including sarcomas and congenital and capillary hemangiomas Ligated umbilical hernia Patent urachus

An omphalomesenteric remnant should be considered in any patient who has a persistent neonatal umbilical lesion. Early diagnosis of this lesion can facilitate treatment and lessen the risk from associated congenital anomalies of the primitive vitelline connection.

Acknowledgements None

Fig. 2: A photomicrograph of the umbilical polyp showing intestinal glands separated by ectatic vessels (H & E, x100)

Funding None

Competing Interests None declared

Reference

1. Steck WD, Helwig EB. Cutaneous remnants of the omphalomesenteric duct. Arch Dermatol. 1964;90:463-470. 2. Nix TE Jr, Young CJ. Congenital umbilical anomalies. Arch Dermatol.1964;90:160-165. 3. McCalla CO, Lajinian S, DeSouza D, et al. Natural history of antenatal omphalomesenteric duct cyst. J Ultrasound Med.1995;14:639-640. 4. Kling S. Patent omphalomesenteric duct: a surgical emergency. Arch Surg.1968;96:545-548. 5. Hejazi N. Umbilical polyp: a report of two cases. Dermatologica.1975;150:111-115. 6. Kittle CF, Jenkins HP, Dragstedt LR. Patent omphalomesenteric duct and its relation to diverticulum of Meckel. Arch Surg.1947;54:10-36.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015

Letter to Editor Spindle Cell Lipoma: An Unusual Lipoma Variant Neelam Sood, Pranshuta Sharma* Department of Pathology, DDU Hospital, New Delhi, India

Dear Sir,

Spindle cell lipoma (SCL) is an unusual lipoma variant that constitutes about 1.5% of all adipocytic tumors. [1] It usually occurs in men, aged 45 to 70 years of age and commonly occurs in the shoulder and neck region. [2] It may be confused with other variants of lipoma on histopathological examination. We report one such interesting case. The patient, a 50 years old man, presented with a soft tissue swelling on the upper back for the last 4 years. The swelling was firm in consistency and mobile. The swelling was excised in toto On gross examination, it was greywhite in color, well encapsulated and firm in consistency. Microscopy showed a population of round to ovoid spindle cells interspersed with short parallel fibrocollagenous bundles surrounded by benign adipocyte cell clusters. Bundles of dense ropy collagen were observed (Fig 1).

Fig. 1a: Microphotograph of the tumor showing adipocytes and spindle cells admixed with a lymphocytic infiltrate (H&E, x100).

There was also an infiltrate of mast cells and lymphocytes. On immunohistochemistry, the tumor was CD34 (fig 2) and S100 positive. This picture was suggestive of spindle cell lipoma. As per the Stanford Diagnostic Criteria, [3] SCL is defined as a predominantly subcutaneous lesion that is composed of mature fat and bland spindle cells. It has a variable amount of adult fat but lipoblasts are absent .Spindle cells are bland looking with scanty cytoplasm and elongated nuclei without mitotic figures. Bundles of dense ropy collagen are typically observed, as in our case. Spindle cells are typically CD34 positive, often extensively and arranged in short parallel arrays, termed â&#x20AC;&#x153;the school of fishâ&#x20AC;? appearance. Even recently, there are reports describing SCL in the usual location, [4] although it is also being reported in other anatomical sites.

Fig. 1b: Microphotograph of the tumor showing ropy collagen and spindle cells (H&E, x400).

*Corresponding author: Dr Pranshuta Sharma, Department of Pathology, DDU Hospital, Hari Nagar, New Delhi, India. Phone: +91-9650079014 E-mail: pranshutasharma.30@gmail.com

This work is licensed under the Creative Commons Attribution 4.0 License. Published by Pacific Group of e-Journals (PaGe)

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Spindle Cell Lipoma

Figure 2 a, b â&#x20AC;&#x201C; Microphotograph showing CD34 positive spindle cells (x100, x400).

Mitotic activity, pleomorphism and the amount of vasculature should be accurately evaluated to rule out malignancy. SCL is composed of bland spindle cells with minimal pleomorphism and mitoses. These bland features are important in differentiating SCL from a liposarcoma. SCL may sometimes be confused with other soft tissue tumors. The features differentiating it from other lipoma variants are described in table 1. Table 1 â&#x20AC;&#x201C; Spindle cell lipoma compared with fibrolipoma and myolipoma Spindle cell lipoma

Fibrolipoma

Myolipoma

Location

Mainly upper back and neck

Variable location

Mainly pelvic area

Morphology

Spindle cell component prominent in a school of fish arrangement.

Spindle cell component absent

Spindle cell with tapered cytoplasm

CD34 status

CD34 positive

CD34 negative

Collagen

Ropy collagen present

Ropy collagen absent

Actin

Actin negative

Actin positive

Spindle cell lipoma is an uncommon variant of lipoma. Variation in the fibrous and myxoid elements can be a source of diagnostic confusion. The presence of CD 34 positive spindle cells and S100 positive adipocytes helps in confirming the diagnosis. Local recurrence after resection is rare. Hence distinguishing it from malignant variants is important.

Acknowledgements None

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Competing Interests None declared

References

1. Fletcher CD, Martin-Bates E. Spindle cell lipoma: A clinicopathological study with some original observations. Histopathology 1987;11:803-17. 2. Enzinger FM, Harvey DA. Spindle cell lipoma.Cancer. 1975;36:1852-3. 3. Rouse RV. Spindle cell lipoma [Internet]. 2007 [updated 2009 July 26]. Available from http://surgpathcriteria. stanford.edu/softfat/spindle_cell_lipoma/ 4. Olaleye O, Fu B, Moorthy R, Lawson C, Black M, Mitchell D. Left supraclavicular spindle cell lipoma. Int J Otolaryngol. 2010 :942152.

Annals of Pathology and Laboratory Medicine, Vol. 02, No. 03, July - September 2015