Alveolar rhabdomyosarcoma involving the heart. An echocardiographic, angiographic and pathologic study.

Abstract

A 12-year-old girl, who had had an above-knee amputation of the left leg for a soft tissue sarcoma six years prior to this admission, presented with syncope, angina, and signs of aortic stenosis. Echocardiography demonstrated a mass arising from the interventricular septum with obstruction of the left ventricular outflow tract and aortic valve. Cardiac catheterization and angiography confirmed these findings. At operation, a tumor was found involving the interventricular septum. Histologically, it was an alveolar rhabdomyosarcoma, and it appeared similar to the previous tumor. The patient was treated by partial surgical resection, cytotoxic drugs and localized radiation therapy and has been followed by serial echocardiography.