Biphasic renal synovial sarcoma with extensive venous tumor thrombosis: A rare presentation

Primary renal synovial sarcoma (SS) is a very rare soft-tissue tumor arising from the kidney. These tumors histologically closely resemble other sarcoma variants. Immunohistochemical and cytogenetic techniques remain the cornerstone in achieving the correct diagnosis. Radical surgical excision is the primary treatment for local tumor control and alleviation of symptoms. Adjuvant chemotherapy, at present, has only a limited role. We report a unique case of a 21-year-old young male with the diagnosis of a biphasic renal SS with extensive venous system thrombosis involving the inferior vena cava, bilateral iliac, and femoral vessels which was managed by radical nephrectomy and tumor thrombectomy followed by adjuvant chemotherapy.