Desmoplastic small pelvic cell tumor: case report and literature review

Abstract

Desmoplastic small round cell tumor (DSRCT), is a type of sarcoma, very rare, with less than 200 reports in the literature worldwide. It is a mesenchymal tumor lesion, with primary involvement of the abdominal cavity and peritoneum, more common in children and young adults, an associated low survival rate at 5 years. Here we report the case of a 22 years old patient with complaints related to an abdominal mass, which underwent complete resection. Final pathology revealed a DSRCT. We describe the primary oncological management, early regional relapse, and rapid progression, reflecting the known poor prognosis of these tumors.