[Primary renal synovial sarcoma. A new entity in the morphological spectrum of spindle cell renal tumors].

We report 2 primary renal synovial sarcoma. These tumors were formerly designated as embryonal cystic sarcoma of the kidney. Most cases are diagnosed between the ages of 20 and 50 years. Some cases show local recurrence after nephrectomy. On gross examination, tumors are large, partially necrotic, and usually contain cysts. Microscopically, tumors are characterized by monomorphic plump spindle cells. The cysts are lined by mitotically inactive epithelial cells without striking cellular atypia. The spindle cells were immunoreactive for EMA, CD56, and sometimes for CD99. They were non-reactive for desmin, actin, S 100, and cytokeratins. The cyst epithelium is cytokeratin positive. The presence of a SYT-SSX gene fusion resulting from the t(X;18) characteristic for synovial sarcoma was demonstrated by reverse transcriptase polymerase chain reaction in both tumors. Primary renal synovial sarcoma is a distinctive tumor entity, which should be considered in renal tumors consisting of spindle cells.