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Synovial sarcomas are tumors that often occur in the proximity of large joints of young adults and adolescents. Primary synovial sarcoma of the kidney is very rare and difficult to diagnose. To date, fewer than 50 cases of primary renal synovial sarcoma (SS) have been reported in literature published in English. There are no established guidelines regarding the management of this tumor given the limited number of cases reported. Although primary surgical resection is the treatment of choice for primary renal synovial sarcoma, the prognosis is poor with surgery alone. Here, we describe a case of a 25-year-old female with primary renal synovial sarcoma that was treated successfully by radical nephrectomy followed by adjuvant chemotherapy. Physicians should be aware of the possibility of malignancy in renal masses and raise the suspicion of synovial sarcoma, especially when patients with renal masses are young adults.
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