| Summary: | Synovial sarcomas are a rare and aggressive subtype of soft tissue sarcomas that typically affects young adults and involves the extremities. Synovial sarcoma of the kidney is a rare and aggressive tumor with a poor prognosis, accounting for only 1% of all renal tumors. The imaging features of this tumor often overlap with those of other renal tumors, and a definitive diagnosis can only be made through immunohistochemical analysis. In this case report, we present the case of a 55-year-old female with left flank pain, who was diagnosed with primary renal synovial sarcoma following a left-sided radical nephrectomy. Despite initial successful surgical intervention, restaging scans showed local recurrence and metastatic disease, which was subsequently managed with 6 cycles of chemotherapy followed by radiation therapy with palliative intent. This case underscores the importance of early detection and aggressive management of rare renal tumors to improve patient outcomes.
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