[Primary renal synovial sarcoma]

The primary renal synovial sarcoma is a rare tumor with a poor prognosis. It may be confused with other types of mesenchymal kidney tumors because of similarities in clinical and histological appearance. About 60 cases have been described in the literature. We report a case of a 66-year-old man presenting a primary synovial sarcoma of the right kidney with a vascular invasion of the inferior vena cava and right renal vein. The diagnosis was confirmed in molecular biology by reverse transcription polymerase chain reaction (RT-PCR) which demonstrated a unique chromosomal translocation t(X;18) with SYT-SSX2 fusion transcripts. We describe here the case with a brief review.