Growth from within : a case of desmoplastic small cell round tumor

Publication Date

2014

Document Type

Research

Abstract

Desmoplastic small cell round tumors are rare tumors primarily affecting young adolescent and adult males which commonly present as intraabdominal masses. Presenting symptoms are non-specific and may only relate to mass effects. Thus, diagnosis mainly relies on histology and immunohistochemistry. A 33-year old male with an enlarging abdominal mass associated with chronic constipation and weight loss. The patient underwent an abdominal computed tomography scan which showed a large highly lobulated heterogeneously enhancing abdominopelvic mass. Colonoscopy showed extraluminal obstruction from the recto-sigmoid to the distal sigmoid colon. Exploratory laparotomy with biopsy of the intraabdominal mass was done which revealed variably sized and shaped, sharply outlined nests of small neoplastic cells surrounded by a prominent desmoplastic and fibromyxoidstroma, consistent with a desmoplastic small round cell tumor. Immunostaining for vimentin, desmin, cytokeratin, epithelial membrane antigen and WT-1 (Wilm'sTumor 1) further confirmed the diagnosis. Optimum treatment modality is yet to be determined however a multimodal approach may provide better survival outcome.

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