Desmoplastic Small Round Cell Tumor: Case Report

Abstract

Introduction: Desmoplastic small round cell tumor (DSRCT) is an extremely rare and aggressive sarcoma of mesenchymal origin that mainly affects the pelvic and abdominal area of the body. DSRCT is rare and occurs more frequently in young white men between the second and third decades of life. Some reports state that, since its discovery, only about 200 cases of DSRCT have been recorded since 1989. Case report: A 20-year-old young adult male presented with abdominal discomfort associated with loss of appetite and weakness for four months, with a weight loss of 10 kg during this period. Findings from complementary examinations included significant hepatosplenomegaly and a pelvic abdominal mass with hepatic and pulmonary nodules. The anatomopathological report of a neoplasm of small round blue cells, associated with the immunohistochemistry available at the service, the epidemiology, and clinical presentation, led to the definition of a presumptive diagnosis of DSRCT and the initiation of immediate systemic therapy. Conclusion: The DSRCT, despite the existing scientific knowledge, and due to its rarity and the limited information on diagnosis and therapy, remains a medical challenge.