Does cytoreductive surgery and hyperthermic intraperitoneal chemotherapy improve survival in children with desmoplastic small round cell tumors? First experiences from Turkey

Objective: Desmoplastic small round cell tumor (DSRCT) is a rare form of highly aggressive sarcoma and despite multimodal therapy, mortality still remains high. The aim of the study is to review our experience in cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in children with DSRCT. Patients and Methods: A retrospective review of patients with DSRCT followed in our institution between January 2020-January 2024 was performed. Demographics, radiological imaging, histopathology results, cytogenetic analyses, chemotherapy/radiotherapy protocols, operative data and outcomes were analyzed. Results: Three patients with DSRCT were identified. The median age was 13.3 years (12-16 years). HIPEC was performed after CRS in two patients, while HIPEC procedure could not be applied in one patient with unresectable tumor. One patient underwent reoperation/re-HIPEC for recurrence after seven months. One patient had the diagnosis after complete removal of the tumor. Patient required peritonectomy and HIPEC as a second operation due to peritoneal recurrence. The patients who underwent CRS and HIPEC are still alive for 33 and 34 months since initial diagnosis, nevertheless the patient who had an unresectable tumor died after 8 months. Conclusion: Cytoreductive surgery and HIPEC may be considered as a safe and feasible treatment option in children with DSRCT in experienced centers.