Primary Renal Synovial Sarcoma

Primary renal synovial sarcoma is a rare malignancy that may present similarly to other renal neoplasms. The diagnosis of synovial sarcoma is performed through the identification of a SYT-SSX gene fusion. Here, we present a case of a primary renal synovial sarcoma in a patient who presented with renal mass initially thought to be renal cell carcinoma until further pathological characterization. After undergoing radical open nephrectomy, the patient developed pulmonary and psoas metastases and was treated with systemic therapy.