dentinogenic ghost cell tumor

Case report

Dentinogenic ghost cell tumour presenting as an asymptomatic gingival swelling in maxillary anterior region: A case report Manuel Suvy* L.K. Surej Kumar** J. Nair Bindu*** ***Menon Varun P.*** Department of Oral and Maxillofacial Surgery - PMS College of Dental Science, Trivandrum, India *BDS, MDS, DNB, MOS RCS(Ed), MFDS RCS (Eng) Oral and Maxillofacial Surgeon, Associate Professor **BDS, MDS, Oral and Maxillofacial Surgeon, Professor and Head ***BDS, MDS, Oral and Maxillofacial Pathologist Professor and Head Dept of Oral and Maxillofacial pathology PMS College of Dental Science, Trivandrum, India ****Junior resident Oral and Maxillofacial Surgeon

Background

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The Dentinogenic ghost cell tumor comes under the spectrum of ghost cell lesions. Uniqueness and rarity of the lesion is evident from the literature.

Here we are presenting a case report of dentinogenic ghost cell tumor which clinically presented as an asymptomatic gingival swelling in the maxillary anterior region along with its diagnosis, management and review of literature.

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Key words: Dentinogenic ghost cell tumor, Maxilla, DGCT, Ghost cell

Suvy M. et al.

Introduction

fig. 1 Clinical photograph showing swelling in the right anterior maxillary region.

Dentinogenic ghost cell tumor is a locally invasive rare neoplasm, seen in any age group from 10 to 90 years, and there is no significant difference between genders. Dentinogenic ghost cell tumor occurring in the mandible and maxilla is called central or intraosseous, whereas in the alveolar mucosa and gingival soft tissues is called peripheral or extraosseous.

The great majority of these tumours are benign and are treated by local resection. The uniqueness of the lesion is quite evident from the literature.

Here we report a case of a dentinogenic ghost cell tumor clinically reported as an asymptomatic gingival swelling in the maxillary anterior region with its diagnosis and treatment.

fig. 2 OPG showing radiolucent lesion involving upper right lateral incisor and canine region.

Case report Case history A 40 year old female underwent clinical examination at the oral and maxillofacial surgery department with complaints of multiple decayed teeth.

Clinical examination revealed an incidental finding of gingival swelling approximately of 2 x 3 cm in size, in relation to 12, 13 region. It was noticed that the swelling was oval shaped, diffuse, extending from the mesial aspect of 11 to the distal aspect of 14, and superiorly it extended into the sulcus (Fig. 1). Overlying gingiva showed a localized gingival fibromatosis. Case history revealed that the patient had noticed the swelling 78 years before, and that there were no symptoms associated with its growth.

Intraoral periapical radiograph showed a well defined circumscribed lesion, of size 1 x 1 cm with sclerotic borders in the region of 12, 13. Vitality test revealed that the teeth involved were vital. Orthopantomograph showed well defined oval shaped radiolucency in relation 12, 13 region (Fig. 2).

Differential diagnosis The patient presented with a history of swelling noticed for the previous 7-8 years in the anterior region of the mandible with no associated symptoms.

Following lesions were considered in the differential diagnosis of this slow

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growing, asymptomatic swelling on the basis of its position, clinical features and radiological features: Peripheral ossifying fibroma, Globulomaxillary cyst, Lateral periodontal cyst, Adenomatoid odontogenic tumour, Squamous odontogenic tumour, Peripheral odontogenic fibroma and Peripheral ameloblastoma.

Peripheral ossifying fibroma is a reactive lesion that only occurs on the gingiva, most commonly in the maxillary anterior region. The peak incidence is between the second and third decades. Women are more likely to be affected than men.

Lesions range in color from pink to red, and the surface may or may not be ulcerated. It is seen as asymptomatic well-circumscribed, pale pink, nodular growth which is non tender and firm to hard in consistency. It has a slight predilection for the maxillary gingiva (1).

The radiographic features may range from no changes to, in rare instances, superficial bone erosions.

Globulomaxillary cysts classically develop between maxillary lateral incisor and cuspid teeth, although occasionally reported between central and lateral incisors. Radiographs typically demonstrate a wellcircumscribed unilocular radiolucency between the teeth. This lesion has a distinct appearance which is an oval, round, or inverted pear-shaped

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radiolucency between the roots of the lateral incisor and cuspid teeth, causing divergence (1). It is usually seen in young adults.

The radiological picture was not similar to that of a lateral periodontal cyst and also they usually present as soft tissue swellings below the interdental papillae. For these reasons lateral periodontal cyst was ruled out from the differential diagnosis.

Adenamatoid Odontogenic tumor is largely limited to younger patients, it has a striking tendency to occur in anterior portions of the jaws and is found twice in the maxilla than in the mandible .Females are affected twice as often as males. Most of these lesions are relatively small. The peripheral forms of the tumor are small sessile masses on the facial gingiva of the maxilla. Clinically these lesions cannot be differentiated from the common gingival fibrous lesions (1). Squamous odontogenic tumor, found in ages ranging from 8 to 74 years, is randomly distributed throughout the alveolar process of maxilla and mandible with no site or sex predilection. A painless or mildly painful gingival swelling is often associated with the mobility of the involved teeth. About 25% of reported patients have had no symptoms. The radiographic findings are not specific or diagnostic (1).

Case report fig. 3 Intraoperative photograph showing the cystic nature of the lesion once the overlying cortex has been removed.

Although peripheral odontogenic tumors are rare when compared with their intraosseous counterparts, a differential diagnosis of a localized gingival mass would be incomplete without their inclusion. Several varieties of these tumors have been reported in the literature, the most common ones being the peripheral odontogenic fibroma and the peripheral ameloblastoma, usually seen in age groups between 23-82 years. The condition usually presents as slow-growing, pink to red, sessile or pedunculated, firm, well-defined, asymptomatic nodules of the attached gingiva. An accurate diagnosis of these lesions is solely dependent on microscopic examination of the excised gingival mass as radiologic features are not seen in most cases (1, 2).

fig. 4 Surgical bed demonstrating the defect and bone loss at the mesial region of canine root, maxillary region.

Surgical management A two sided full thickness trapezoidal mucoperiosteal flap was elevated, extending within the area from tooth 15 to 21. The lesion was identified in relation to 14, 13, and 12. Clinically it was seen that the labial cortical plate was perforated at some levels.

The cystic lining was clearly evident (Fig. 3), once the overlying bone was removed .Following the enucleation it was seen that tooth13 had mild root resorption on the mesial aspect (Fig. 4). The excised specimen was sent for histopathologic examination.

moderately collagenous connective tissue with odontogenic epithelium, clusters of ghost cells undergoing

dystrophic calcification and eosinophilic areas suggestive of dentinoid (Fig. 5), the latter were seen in close contact

Diagnosis The

soft

tissue

section

showed

fig. 5 Moderately dense collagenous stroma showing large areas of dentinoid and interconnecting strands of odontogenic epithelium. (H & E X100).

fig. 6 Areas of dentinoid with ghost cells undergoing calcification and odontogenic epithelium (H & E X 400).

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fig. 7 Clusters of ghost cells showing calcification seen in association with odontogenic epithelium and dentinoid (H & E x 400).

fig. 8 Proliferating odontogenic epithelium (H & E X 400).

fig. 9 Van Gieson stain showing (x 400). a) ghost cells staining yellow. b) Surrounding dentinoid staining pink. c) odontogenic epithelium.

with the odontogenic epithelium and ghost cells (Fig. 6, 7). The odontogenic epithelium was arranged in interconnecting cords and islands and was composed of low columnar and spindle cells (Fig. 8). In some portions of the tumour, irregular areas of calcified material were seen. Adipose cells in the connective tissue and mature bone in the periphery were also observed.

The histopathological picture gave an impression of Dentinogenic ghost cell tumor. It was decided to study more sections of the excised specimen using special stains to confirm the diagnosis. Van Gieson staining was done and the ghost cells took a yellow stain and dentinoid pink (Fig. 9). The asymptomatic swelling was confirmed as Dentinogenic ghost cell tumor (DGCT).

Discussion The rarity of the occurrence of DGCT as well as the cystic appearance of the lesion mislead us, thus not including it in our differential diagnosis. As for the classification of ghost cell odontogenic tumours (3), only CCOT type 1 is a cystic form and not the dentinogenic

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Case report ghost cell tumour, so the biopsy report came quite as a surprise. Hence this appears to be a unique case of DGCT which presented in cystic form.

DGCT currently comes under the spectrum of ghost cell lesions, and Calcifying Odontogenic Cyst (COC) is one of the earliest described ghost cell lesion: it was first described by Gorlin et al. in 1962 (4). It represents about 1% of all odontogenic cysts. COC tends to occur around the third decade of life, though patients age ranges from 7 to 82 years (5, 6). It occurs equally in the maxilla and mandible, usually anterior to the first permanent molar, though it has a predilection for the maxilla in the younger age range.

It occurs equally in males and females.

It exists in two entities: cystic tumor or solid neoplasm (5, 6) In 1981, Praetorius et al. proposed a classification which resolved the dilemma between the cystic or neoplastic nature of calcifying odontogenic cyst. He recognized four different histological patterns of COC and classified them as type 1A (simple unicystic), type 1B (odontome-producing), type 1C (ameloblastomatous proliferating) and type 2 (dentinogenic ghost cell tumour) (5).

According to a recent WHO classification of odontogenic tumours, COC was re-named as calcifying cystic odontogenic tumour (CCOT).

Ghost cell odontogenic tumours were classified into three entities (7). The first entity CCOT is characterized by a well-defined, cystic, painless, slowly growing neoplasm that rarely relapses.

The second, the DGCT, is a locally invasive neoplasm which shows more aggressive behavior and recurrence.

The third entity is a malignant counterpart of CCOT and DGCT, named as ghost cell odontogenic carcinoma (GCOC) (3).

Dentinogenic ghost cell tumor (DGCT) is an extremely rare odontogenic tumor. According to Buchner’s (8) research on 215 cases of intraosseous calcifying odontogenic cysts, calcifying odontogenic cysts (COCs) account for 1-2% of all odontogenic tumors and only 2-14% of COCs are solid tumors, the latter were considered to be DGCTs. Thus, based on even a large sample size of patients that may have DGCTs, it remains a rare finding.

Dentinogenic ghost cell tumor (DGCT) was first described by Fejerskov and Krogh (9) in 1972. They had used the

term “calcifying ghost cell odontogenic tumor”; but in 1981, Praetorius et al.

(5) suggested the term “dentinogenic ghost cell tumor”.

Shear (10) in 1983 gave the term “dentinoameloblastoma” because of its similarities with ameloblastoma and dentinoid production. In 1986, Ellis and Shmookler (6) proposed the term “epithelial odontogenic ghost cell tumor” as they thought that the ghost nucleated keratinizing cell was the most distinctive histopathological feature. It was Hong et al. (11) in 1991 who suggested the term “epithelial odontogenic ghost cell tumor” as characteristic of these neoplasms are the odontogenic epithelial proliferations with some inductive activity and the formation of ghost cells.

In 2003, Li and Yu (12) suggested the term “odontogenic ghost cell tumor” which was originally described by Ellis (13) in 1999. This term emphasized its origin, neoplastic nature, and most striking histopathological features. In 2005, the World Health Organization (WHO) (7) retained the term dentinogenic ghost cell tumor as initially proposed by Praetorius et al.

(5). The WHO defined this tumor as “A locally invasive neoplasm characterized by ameloblastoma-like islands of epithelial cells in a mature connective tissue stroma. Aberrant keratinization may be found in the form of ghost cells in association with varying amounts of dysplastic dentin” (7).

DGCTs have occurred in patients ranging from 10-92 years of age, with an average age of occurrence of 59 years (14). Two variant forms of DGCT are known to occur; DGCT type1 Central (in the mandible or maxilla) as seen in our case and DGCT type 2 Peripheral (in the gingival; soft tissues or alveolar mucosa). The peripheral variant is the less aggressive of the two types (3).

The presence of ghost cells within the proliferative odontogenic epithelium is the essential characteristic for the diagnosis of ghost cell tumours.

However, the ghost cells alone are not sufficient for the diagnosis of DGCT nor is it pathognomonic. Ghost cells may also be observed in other lesions such as odontomas, ameloblastomas, and ameloblastic fibro-odontomas (15). The latter tumors were eliminated from the histopathological differential diagnosis by the absence of a cellular primitive ectomesenchyme resembling

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dental papilla ,and the presence of dentinoid confirmed the lesion as dentinogenic ghost cell tumour.

The treatment involves a surgical excision with a long-term follow up and the recurrence depends on completeness of cyst removal (16).

Howerver, in our literature search we came across a case of DGCT in the left body of the mandible, which underwent segmental resection and was reconstructed using an absorbable collagen sponge, rhBMP-2 and a titanium plate and mesh (17).

The prognosis of DGCT is determined by the treatment provided. Numerous cases in the literature have been reported to recur, mainly owing to inadequate excision. The retreatment of such cases was found to be totally curative. A review by Kasahara et al demonstrated the rate of recurrence of central DGCTs following resection to be approximately 44% (18). Wide local resection is the recommended mode of treatment for DGCT (19).

Till 2009 approximately 32 cases of DGCT have been reported in English literature (19).

The malignant transformation of the DGCT after several recurrences has been documented (20). The present case is under routine follow up for the past two years and there is no evidence of any recurrence.

Conclusion Ghost cell tumours are relatively rare and DGCT, being a part of the spectrum is even rarer, and this is the reason for not including it in the initial differential diagnosis. The uniqueness of DGCT is evident in the discussion, as it has been known by a multitude of definitions and multiple classifications.

Thus we have presented a case of DGCT with cystic features presenting as an asymptomatic gingival swelling in the anterior maxillary region.

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