Desmoplastic small round cell tumor of the kidney with bone metastasis: A case report

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive sarcoma that predominantly affects adolescent and young adult males. DSRCT typically arises from the serosal surface of the abdominal cavity with a high propensity for metastatic disease and, subsequently, poor prognosis. The diagnosis is often challenging due to its nonspecific clinical presentation and overlapping imaging features with other abdominopelvic entities. Consequently, imaging, particularly computed tomography (CT) and positron emission tomography-computed tomography (PET-CT), plays a central role in the initial evaluation. Ultimately, a definitive diagnosis requires histopathologic confirmation, given that DSRCT shares imaging and histologic characteristics with other round cell neoplasms. In this article, we report a case of a primary DSRCT of the kidney in a 23-year-old male, highlighting the clinical presentation, imaging features, and aggressive course of this tumor.