Abstract
Synovial sarcoma is a rare mesenchymal tumor, and its occurrence as a primary renal tumor is exceedingly rare. We are presenting a case of renal synovial sarcoma with lung involvement in a 47-year-old female patient who initially presented with typical renal symptoms, including blood in urine and left flank pain. Imaging revealed a large renal mass with extension into the renal vein and metastatic nodules in the lungs. Histopathological examination and genetic analysis confirmed monophasic synovial sarcoma with SYT-SSX2 translocation. Treatment included radical nephrectomy followed by systemic chemotherapy with doxorubicin and ifosfamide. Despite the initial response, the disease progressed, leading to fatal complications. This case highlights the diagnostic challenges, limited treatment options, and poor prognosis associated with primary renal synovial sarcoma.