Key result
Case report describes a rare primary desmoplastic small round cell kidney tumor with bone metastasis.
Why the study?
Desmoplastic small round cell tumor is a rare, highly aggressive sarcoma whose diagnosis is challenging due to nonspecific clinical presentations and overlapping imaging features with other abdominopelvic entities.
Case Report (n=1)
Highlights the clinical presentation, imaging features, and aggressive course of a rare primary DSRCT of the kidney.
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.
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Smith et al. (2026) conducted a case report in Desmoplastic small round cell tumor (DSRCT) of the kidney (n=1). A 23-year-old male presented with a rare and aggressive primary desmoplastic small round cell tumor of the kidney with bone metastasis.
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