Cytoreductive Surgery Followed by Hyperthermic Intraperitoneal Chemotherapy in DSRCT: Progress and Pitfalls

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) is an approach for local control of desmoplastic small round cell tumor (DSRCT). DSRCT is a rare sarcoma which presents with multiple intra-abdominal masses. Overall survival has been 15-30%. Because of the poor prognosis of DSRCT, novel treatment strategies were necessary. Cytoreductive surgery (CRS) and HIPEC has been recently trialed as part of multimodality therapy in DSRCT. CRS and HIPEC allows complete resection of the sometimes hundreds of intra-abdominal tumor implants, followed by the delivery of hyperthermic cisplatin for 90 min at approximately 41 °C. HIPEC is thought to enhance microscopic control of abdominal DSRCT, after surgical resection, and prevent or prolong recurrence. Here, the background of DSRCT, the multimodal treatments available, and the progress and pitfalls of CRS and HIPEC in DSRCT are reviewed.