Peritoneal sarcomatosis in pediatric malignancies

Peritoneal sarcomatosis (PSC) is defined as peritoneal involvement of multiple sarcomatous tumors. Desmoplastic small round cell tumors (DSRCT) and rhabdomyosarcomas are the most common pediatric PSC cases. PSC has been treated with chemotherapy and mainly palliative surgery, but long-term outcome has been poor. New imaging technologies have improved the evaluation of disease extent and patterns of peritoneal dissemination, and cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC) is being evaluated as a treatment option to prolong remission in pediatric patients. We will review the clinical characteristics, potential biologic mechanisms, radiographic characteristics, and potential therapies for pediatric PSC patients.