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A rare cause of bilateral pleural effusion – desmoplastic small round cell tumor

Desmoplastic small round cell tumor (DSRCT) is a rare, extremely aggressive and malignant tumor predominantly affects young adolescent males and typically presents as a large intra-abdominal mass. However, tumor arising from other body sites are also reported in the literature. Histology and immunohistochemistry play an important role in the diagnosis and differentiating this rare tumor from other round cell tumors. A multidisciplinary approach consisting of a combination of surgery, chemotherapy and radiation therapy is the treatment of choice as there is no standard therapy.  We report a case of DSRCT of pleura presenting as bilateral pleural effusion in a young adolescent male who was treated with both surgery and chemotherapy. However patient succumbed to death after one year of diagnosis.