Synovial Sarcoma of The Kidney: An Update

A Synovial sarcoma (SS) is an uncommon mesenchymal tumour entity which accounts for 5% to 10% of soft tissue sarcomas (STS). Primary renal synovial sarcoma (PRSS) is a very rare, rapidly growing tumour, which is associated with the potential for the development of metastatic disease. The main prognostic factors of PRSS include the size of the tumour, the histopathology grade, as well as translocation t (X; 18) (p11.2; q11.2) (fusion of SYT gene -chromosome 18- with SSX genes (1, 2 or 4)-chromosome X) is recognised as the commonest pathognomonic sign. Aggressive surgical resection of the tumour with complete excision of the together with concomitant regional lymphadenectomy is the treatment of choice for majority of PRSSs, while additional en bloc resection of the adjacent affected organs is often undertaken. At the moment, the role of pre-operative or post-operative chemotherapy has remained equivocal. The prognosis of patients with PRSS has tended to be poor, as the 5-year survival rate has been documented to vary from 20% to 30% in reported cases and studies and further deterioration in the outcome pursuant to treatment has been documented in association with the pathology examination finding of a high mitotic activity. Local recurrence even after complete radical excision of the kidney tumour has remained the most frequent recurrence which eventually emanates in the development of distant metastases and death of patients.