Management of Desmoplastic Small Round-cell Tumors in Children and Young Adults

Desmoplastic small round cell tumor (DSRCT) is a rare type of sarcoma which is primarily abdominal in origin. Less than 200 cases have been reported. DSRCT presents with multifocal tumors believed to begin in the peritoneal surfaces of the abdominal cavity. Up to hundreds of tumors can be found in the abdominal cavity. Adolescent males are primarily affected. Because of the rarity of the tumor and the unusually aggressive presentation, treatment is challenging and has not been standardized. Here we summarize the clinical presentation, diagnosis and management options of this rare tumor.