Desmoplastic Small Round Cell Tumors- Case series of a Rare Tumor and Review of Literature

Abstract

Desmoplastic small round cell tumor (DSRCT) is an uncommon and aggressive malignancy with male predominance. It commonly occurs in the abdominal cavity and is usually diagnosed with help of histopathology. It shows a characteristic EWSR1-WT1 gene fusion in most cases. We hereby report a series of 3 cases of DSRCT that we encountered at our institute. All of them presented in an advanced stage as quoted in the previously available literature. The ideal treatment should include a multidisciplinary team. The chemotherapy regimen used is usually Ewing’s sarcoma protocol of multiagent chemotherapy. Surgical approaches which include cytoreduction and intra-peritoneal hyperthermic chemotherapy are the procedure of choice but rarely feasible. Newer clinical trials of targeted therapy are in progress for improving the treatment and thereby the prognosis of DSRCT.