Extensive Intra-abdominal Desmoplastic Small Round Cell Tumor With Hepatic Metastases: A Report of a Rare Case

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive malignancy predominantly affecting young male patients, typically originating from the abdomen or pelvis, with a poor prognosis. Since the first description as a distinct clinical entity in 1989, approximately 850 cases have been reported in the literature. All cases share the chromosomal translocation t(11;22)(p13;q12), leading to the formation of the EWSR1-WT1 fusion gene. DSRCT characteristically arises in the abdomen or pelvis and carries a poor prognosis despite multimodal therapy. We report a rare and extensive case of abdominopelvic DSRCT with liver and peritoneal metastases in a 17-year-old Moroccan male, who presented with a progressive abdominal mass, pain, and hepatomegaly. The diagnosis was initially challenging due to the non-specific clinical presentation. The final diagnosis was established through liver biopsy and immunohistochemical analysis. The patient is currently undergoing treatment with a multidrug chemotherapy regimen.