ABSTRACT
Synovial sarcomas occur predominantly in the extremities. Primary renal synovial sarcoma is a rare entity. Very few cases have been reported in the literature. Clinical and radiological features are similar to renal cell carcinoma with the diagnosis being established after surgery based on histopathology, immunohistochemistry, and chromosome studies. There are no established guidelines on the role of adjuvant treatment in the management of this disease. We herein present a series of 3 cases managed at 2 institutions. In the current series, all patients had venous thrombus, and surgery was the mainstay of treatment. One patient received neoadjuvant chemotherapy after a preoperative biopsy which was done as she did not respond to chemotherapy for a presumptive diagnosis of Wilm's tumor.