Intra-Abdominal Desmoplastic Small Round Cell Tumor (DSRCT) and the Role of Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Review

• Published in: Current oncology (Toronto) Vol. 30; no. 4; pp. 3951 - 3963
• Main Authors: Reijers, Sophie J M, Siew, Caroline C H, Kok, Niels F M, Honoré, Charles, van Houdt, Winan J
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI 31.03.2023; MDPI AG
• Subjects: Combined Modality Therapy; CRS; cytoreductive surgery; desmoplastic small round cell tumor; Desmoplastic Small Round Cell Tumor - drug therapy; Desmoplastic Small Round Cell Tumor - pathology; DSRCT; HIPEC; Humans; Hyperthermic Intraperitoneal Chemotherapy; Male; Peritoneal Neoplasms - drug therapy; Review; Sarcoma; desmoplastic small round cell tumor; DSRCT; hyperthermic intraperitoneal chemotherapy; HIPEC; CRS; cytoreductive surgery
• ISSN: 1718-7729; 1198-0052; 1718-7729
• DOI: 10.3390/curroncol30040299

Desmoplastic small round cell tumor is a very rare and highly aggressive soft tissue sarcoma, usually presenting with multiple intra-abdominal tumors in young males. Patients present with advanced disease and the overall survival is dismal. Multiple studies report relatively favorable outcomes with multimodal treatment consisting of chemotherapy, surgery and radiotherapy. If resection is feasible, complete cytoreductive surgery is the cornerstone of surgical treatment. The benefit of hyperthermic intraperitoneal chemotherapy in addition to cytoreductive surgery is unclear, and few studies have evaluated this option. We sought to identify the role of hyperthermic intraperitoneal chemotherapy in patients with intra-abdominal desmoplastic small round cell tumor. Our review of the available literature revealed no clear survival benefit in performing hyperthermic intraperitoneal chemotherapy after cytoreductive surgery.