[Malignant fibrous histiocytoma of the heart--a case report and review of the literature].

Abstract

Malignant fibrous histiocytoma (MFH) has been rarely reported as a primary tumor of the heart. We present a case of a primary intracardiac MFH and review the 28 previous reports of this rare cardiac tumor. A 48-year-old woman underwent an operation for congestive heart failure (CHF) due to a left atrial tumor. A large multinodular tumor attached to the posterior wall of the left atrium and extended into the mitral annulus and the posterior leaflet of the mitral valve. The tumor was resected incompletely and mitral valve replacement was performed. Pathological diagnosis was MFH. The patient did well in the postoperative period, but she again presented with symptoms of CHF four months later. Chest CT and two-dimensional echocardiogram demonstrated a recurrent tumor of the left atrium. Since the tumor grew rapidly despite chemotherapy, a reoperation was performed to remove it. Nevertheless, as the tumor was found to have extended into the pericardial cavity and posterior mediastinum, only partial resection was possible. After a protracted operation, the heart failed to sustain the circulation and the patient died. The prognosis of intracardiac MFH was poor in spite of surgery, radiation and/or chemotherapy. However, repeated surgical treatments can relieve symptoms of CHF and may prolong the life of patients.