Primary synovial sarcoma of the heart. A clinicopathologic study of one case and review of the literature.

Abstract

Primary synovial sarcomas of the heart are aggressive and extremely rare tumors. Only thirteen cases have been reported previously. We report the fourteenth case and review the previous ones. Our patient is a 45 year-old man who presented with congestive heart failure, which was related to a right atrial pedunculated tumor. The tumor was resected completely and the patient has remained disease-free after 5 years. The histopathologic examination and immunohistochemistry study confirmed the diagnosis. Thirteen cases are reported in the literature (11 men, 2 women) with a median age of 37.38 years (13 to 53 years). Most of the tumors occur in the right side (in 8 cases) particularly in the right atrium (in 5 cases). Ten patients died, seven within one year of diagnosis. However, in our case, the patient is still alive and free of disease.