Desmoplastic Small Round-Cell Tumor in a Young Indigenous Australian Man: A Case Report

Desmoplastic small round-cell tumor (DSRCT) is a rare and highly aggressive neoplasm of children and young adults that primarily arises from the abdomen with widespread peritoneal involvement and is characterized by distinct clinicopathologic features. Therapeuticmanagement of DSRCT remains challenging, and no standard therapy is currently described in the literature. Despite the combination of aggressive treatmentssuchaschemotherapy,debulkingsurgery,and whole abdominal radiation, the prognosis of DSRCT remains extremely poor. There are fewer than 200 cases reported in literature. We report the first case of an Australian-indigenous man with DSRCT and also describe our treatment approach for this rare cancer.