Intraperitoneal Rupture of a Synovial Sarcoma of the Kidney.

ABSTRACT

Primary renal synovial sarcoma is an aggressive, extremely rare disease. Nearly all reported cases are characterized by SYT-SSX gene translocation (X;18)(p11;q11). We describe the case of a 43-year-old woman who presented with an intraperitoneal rupture of this rare sarcoma followed by a right radical nephrectomy. Follow-up imaging 1 month after surgery revealed peritoneal carcinomatosis. She began systemic chemotherapy with doxorubicin and dacarbazine, progressed after 3 months, and is currently receiving single-agent ifosfamide. Only one instance of intraperitoneal rupture has been reported previously. This case report contributes to the characterization of this rare disease.