Management of desmoplastic small round cell tumor

Abstract

Desmoplastic small round cell tumor (DSRCT) is a soft tissue sarcoma of mesenchymal cell origin, presenting with multiple intra-abdominal tumors. The organ or specific tissue type of origin is unknown. It is rarely isolated to one tumor implant in the abdomen and in most cases presents with dozens to hundreds of abdominal peritoneal tumors. One very large dominant mass is usually present in the omentum with the second and third largest conglomerate of tumors being in the pelvis and right diaphragm peritoneum, respectively. Despite the often massive amount of abdominal tumors, symptoms of bowel obstruction are rare. Ascites may be present. In late stages, pleural effusions, pleural implants, mediastinal adenopathy, supraclavicular adenopathy, or bone metastasis may be present. With this challenging disease, multidisciplinary therapy as well as aggressive surgery is warranted. This review will address DSRCT treatment options and discuss outcomes.