Desmoplastic Small Round Cell Tumor: A Case Report

Abstract

Introduction Desmoplastic small round cell tumor (DSRCT) is an ultra-rare and highly aggressive mesenchymal tumor that primarily affects the peritoneum, and to a lesser extent, the visceral organs in the abdominal cavity. This study presents a case of a patient who has been diagnosed with DSRCT and has been managed with VAC-IE chemotherapy. Case presentation A 43-year-old male presented with a complaint of epigastric pain for 20 days but no other clinical symptoms. A CT scan and an abdominal ultrasound showed multiple lesions in the peritoneum, and biopsies were taken from all the sites. Histological analysis revealed nests of small blue round cells surrounded by an abundant stroma. Immunohistochemistry results confirmed the disease to be a desmoplastic small round cell tumor. Conclusion This disease is yet to be fully understood, and the response of our case to VAC-IE chemotherapy has shown promising outcomes. This form of treatment can be further studied to establish and help in finding a treatment with a better prognosis.