Barw Barw Medical Journal 2960-1959 2023 12 28 Desmoplastic Small Round Cell Tumor: A Case Report 10.58742/etbs9n84 eng Rebaz Haji Ali Hiwa Cancer Hospital Centre, Sulaimani Directorate of Health, Sulaimani, Kurdistan, Iraq. rebaz.ali12@gmail.com Shano M. Ali Hiwa Cancer Hospital Centre, Sulaimani Directorate of Health, Sulaimani, Kurdistan, Iraq. shano.ali@gmail.com Rawa M. Ali Smart Health Tower, Madam Mittrand, Sulaimani, Kurdistan, Iraq. rawa.ali@gmail.com Soran H. Tahir College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaimani, Kurdistan, Iraq. soran.tahir@gmail.com Shad Nasir Mohammed College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaimani, Kurdistan, Iraq. shad.mohammed@gmail.com Dana T. Gharib Gastroenterology and Hepatology Teaching Hospital, Sulaimani, Kurdistan, Iraq . dana.gharib@gmail.com Zana Baqi Najmadden Research Center, University of Halabja, Halabja, Kurdistan, Iraq. zana14@gmail.com Hoshmand R. Asaad Gastroenterology and Hepatology Teaching Hospital, Sulaimani, Kurdistan, Iraq . hoshmand.asaad@gmail.com Deari A. Ismaeil College of Medicine, University of Sulaimani, Madam Mitterrand Street, Sulaimani, Kurdistan, Iraq. deari34@univsul.edu.iq Omar H. Ghalib Hawramy Smart Health Tower, Madam Mittrand, Sulaimani, Kurdistan, Iraq. omar.hawrami@gmail.com 2023 11 10 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.