Research Paper
E-ISSN No : 2455-295X | Volume : 2 | Issue : 7 | July 2016
GLOMUS OR GLOMANGIOMYOMA. IS IMMUNOHISTOCHEMISTRY ONLY WAY TO SUBCLASSIFY? - A RARE CASE AT THE SITE OF STOMACH 1
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Dr. Ashima Chugh | Dr. R. K. Saran | Dr. S. Saluja 1 2
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Department of Pathology, GB Pant Hospital. Department of Gastro Surgery, GB Pant Hospital.
ABSTRACT Glomangiomyoma are glomus tumour that display focal or partial smooth muscle differentiation, now comes under broad category of perivascular myoid tumours. They are usually benign & mesenchymal in origin. They are frequently encountered in the deep dermis or subcutaneous tissues of the upper or lower extremity. Rarely, they have been found in unexpected sites such as the stomach, small bowel, rectum, urinary tract, lung, cervix etc. We report an unusual & rare glomangiomyoma of stomach in 32 year old female which presented as mass lesion in the distal stomach. The surgical treatment was done and the patient is on follow up. KEYWORDS: Glomangiomyoma, stomach. Introduction The glomus bodies are specialized neurovascular organs of the skin involved in thermoregulation. They are basically act as Arterio- venous shunt through a short segment of thick wall area having plump cuboidal endothelial cells. Glomus cells are scattered epithelial like cells in between the circular and longitudinal muscle fibers. Glomus tumor are suggested to be arising from these bodies. Depending on component and proportions of glomus cells, smooth muscle cells and vascular structure, these essentially benign tumours are called as 1) Glomus, 2) Glomangioma and 3) Glomangiomyoma. Glomangiomyoma mostly occur subcutaneous tissues of the upper or lower extremity, especially the palms, soles, and nail beds.1 Rare sites may be stomach, small bowel, rectum, urinary tract, lung, cervix, vagina, mediastinum, trachea, oral cavity, bone, mesentery, heart, and lymph nodes2,3. The first case of gastric glomus tumor was reported way back in 1951 by Kay et al4. REPORT OF A CASE A 32 year old female came to gastrointestinal surgery OPD & complained of recurrent episodes of hematemesis associated with malena. She was admitted to private hospital & was given blood transfusion & was referred to our hospital. Her last episode of malena was around 1 month back. UGI endoscopy was done showed a 5 cm submucosal mass with central umbilication in antrum of stomach. CECT abdomen showed a well defined soft tissue of 4 X 2.7 X 2.8 cm eccentrically located arising from antropyloric region with central umbilication. A provisional diagnosis of Gastric GIST was made. The patient was operated & specimen on gross examination, showed a partial gastrectomy specimen measuring 5 X 4.5 X 2 cm. Mass was mainly located in submucosa with a central ulceration with raised margins. Cut surface showed a firm whitish mass of 1cm.
centage of each component to sub-classify it. Also, problem arises when HE section did not clearly identify smooth muscle differentiation as in our case. Only focal presence of epithelioid like cells noted. The first differential diagnosis based on imaging is GIST. GIST is the most common mesenchymal tumor in the stomach and should be kept as first D/D. Morphology histological features of GIST are classical and did not comes under D/D with Glomus group of tumour. The second differential comes is paraganglioma. The cells are arranged in a characteristic alveolar or Zellballen pattern with rich thin-walled blood vessels in stroma which can easily be confused with glomus tumor. The cells show immunopositivity for cytokeratin, S-100 protein, synaptophysin, chromogranin A, and neurone specific enolase, but negative for SMA. Since patients have no specific clinical and imaging findings, it is difficult to diagnose before operation. Histopathological features of small, uniform and round tumor cells surrounding capillaries and strongly positive for SMA, are helpful to make its diagnosis. Our case is unique because of its unusual location. Conclusion Glomangiomyoma is a tumor that mainly affects the lower limbs, but in rare cases, as reported, can affect other organs, such as the stomach, pancreatic head, kidney etc. GIST & paragangliomas are considered in the differential diagnoses of patients with epigastric pain, post-prandial vomiting and weight loss. The surgical treatment with enucleation after diagnosis is the treatment of choice. Photographs
Microscopy clusters of benign small round to oval cells with clear cytoplasm were seen arranged around thin vascular channels. Mitosis was infrequent. Focal epitheloid differentiation was noted. These cells have moderate and pale eosinophilic cytoplasm, did not clearly show morphology of smooth muscle differentiation on H&E (arrow of Fig.) The epicentre of tumor is in the submucosa and the tumour was pushing into the muscularis propria. Serosa was free from tumor along with both the resection margins. Atypical mitosis, or other features of malignancy like > 2 cm size or mitosis >5 / 50 HPF were not noted. Immunohistochemistry showed positivity for SMA, vimentin and caldesmon. Most of the cells have taken moderate intense staining for SMA. Focal positivity of caldesmone noted. They were immunonegative for CD117, CD34, synaptophysin & chromogranin. MIB index was less than 1%. Reticulin stain showed reticulin fibres surrounding group of cells rather than individual cells. Discussion Gastric glomus tumor is a rare benign perivascular myoid cell tumor arising from the glomus body. Its clinical features are nonspecific comprising of epigastric pain and bloody stool. Gastric glomus tumor are more common in women than in men with a female prepondance. The median age of the patient is 45 years (range 28-79 years). Most commonly these tumors are located in antral part of stomach. The tumor is usually solitary but cases of multiple gastric glomus tumors have been reported.5Glomangiomyomas are glomus tumors with relative focal or diffuse presence of smooth muscle cells. Literature did not specify the exact per-
Photograph 1-Small round cells with round to oval nuclei & thin walled dilated vessels(H&E,10X)
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International Educational Scientific Research Journal [IESRJ]
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Research Paper
E-ISSN No : 2455-295X | Volume : 2 | Issue : 7 | July 2016
Photograph 2-A small area showing focal myomatous differentiation (H&E,20X)
Photograph 3-The tumor cells show immunopositivity for SMA.(10X)
Photograph 4-Tumor cells show focal immunopositivity for hcaldesmon(H&E,10X) REFERENCES 1.
Enzinger FM, Weiss SW. Perivascular tumors. In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors.3rd ed. St Louis, Mo:The CVMosbyCo;1995:701–713.
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Salima H, Modlin IM, West AB. Multiple glomus tumours of the stomach with intravascular spread. Am J Surg Pathol.1992;16:291–299.
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Geraghty M, Everitt NJ, Blundell JW. Glomus tumour of the small bowel. Histopathology 1991;19:287–289.
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Chou KC , Yang CW, Yen HH. Rare gastric glomus tumor causing upper gastrointestinal bleeding, with review of the endoscopic ultrasound features. Endoscopy 2010; 42 Suppl 2:E58-E5
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Alempijevic T, Knezevic S, Knezevic D, Ostojic S, Stojakov D, Micev M, Tomic D, Krstic M. Gastric multicentric glomangioma: a case report of this rare cause of abdominal pain. Med Sci Monit 2008; 14: CS5-CS8
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International Educational Scientific Research Journal [IESRJ]