Cytoredutive Surgery and Chemohyperthemia for Desmoplasic Small Round Cell Tumor in Pediatric Patient: Case Report

Abstract

Introduction: Desmoplastic small round cell tumor (DSRCT) is rare and highly aggressive mesenchymal tumor. Objective: Case report of a 7 y-o boy, diagnosticated with a DSRCT, treated in the Pediatric Service at National Cancer Institute José Alencar Gomes da Silva. Case report: He presented with abdominal pain, abdominal mass, ascites, fever and slimming. computer tomography showed a hypodense tumor on IV hepatic segment, volumous ascitis, tumoral mass in right hemithorax. He was submitted to needle biopsy with histopatologic result as DSRCT staged as IV. The patient was submitted to sistemic chemotherapy with complete response on thoracic tumor e abdominal tumor reduction, with stable pelvic lesions. an cytoredutive surgery with cisplatin hyperthermic intraperionteal chemotherapy. He received abdominal radiotherapy and chemotherapy. He presented with tumor progression and death after 14 months. Conclusion: Cytoredutive surgery and Hyperthermic intraperitoneal chemotherapy permitted a temporary disease control with a good quality of life.