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Year : 2020  |  Volume : 6  |  Issue : 1  |  Page : 87-89

Clear cell sarcoma of rectum with liver and lung metastasis in a young female: A rare case report with review of literature

Assistant professor, State Cancer Institute, IGIMS, India

Correspondence Address:
Kunal Kishor
Dept. of Radiation oncology, State Cancer Institute, IGIMS, Patna
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Source of Support: None, Conflict of Interest: None

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INTRODUCTION: Clear cell sarcoma like tumor of the gastrointestinal tract (CCS-GI) is an extremely rare type of soft tissue sarcoma with aggressive behavior, which exhibits morphological, immunohistochemical and ultrastructural similarity with malignant melanoma. It is rarely localized in the intestine and the natural history of this tumor is not yet clear[1]. To date, less than 42 cases of CCS-GI have been described in the literature, as far as the best of my knowledge. CASE PRESENTATION: A 20 year nulliparous female presented with something coming out during straining for defecation and bleeding per rectum since 3 months. Patient was also complaining of constipation off and on. The patient underwent contrast enhanced MRI Pelvis, which revealed mass in anorectal region, 6.5x 3.4cm in size at left pararectal wall which was touching muscularis externa at 3 O’clock. There was mid perirectal mesorectal lymph nodes, 2-4 cm in size. Biopsy from anorectal mass was sent for HPE, which was reported as malignant melanoma. In morphology, tumour cells were relatively large, ovoid to epithelioid with pale eosinophilic to clear cytoplasm. Nuclei were centrally located. Necrosis was present. Immunohistochemistry was positive for S100 and Melan-A. The tumor was immunonegative for CK, HMB-45, CD1a,CD68, CD21 and CD23. Ki-67 was70%. Final impression was Clear cell sarcoma like tumor of the gastrointestinal tract (CCS-GI). CONCLUSION: Herein we present a case of CCS-GI, with discussion on its clinical and pathological features and review of the literature on the subject.

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