To update epidemiological, diagnostic, and therapeutic data on primary kidney synovial sarcoma. A total of 96 papers were reviewed; the average age at presentation was 38.614.2 years; the most common site of the tumor was the right kidney; hematuria and pain were the most often reported symptoms at diagnosis. The cytogenetic method was utilized to make a final determination. In 37.8 percent of cases, an oncogene was discovered, with most patients having SS18-SSX fusion. Surgery is the preferred treatment, with adjuvant chemotherapy, most often ifosfamide-based doxorubicin or epirubicin. The median overall survival time was 34 months. Mortality was recorded in 29 percent of the cases, and the recurrence rate was 39.8 percent. Patients having metastases at the time of diagnosis had a higher risk of mortality.

Primary RSS is more common in young males. RSS frequently manifests with symptoms and at an advanced stage. The most frequent treatment is surgery, with chemotherapy used for advanced or recurring cases.

Reference: https://link.springer.com/article/10.1007/s11934-021-01038-w

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