Nongenital pelvic leiomyosarcoma metastatic to the heart

A patient who had a 5-year history of a low-grade nongenital pelvic leiomyosarcoma was evaluated for worsening dyspnea, hypertension, and jugular venous distension. An echocardiogram revealed a large right atrial mass. At surgical exploration, metastatic leiomyosarcoma was found within the inferior vena cava extending from below the renal veins up into the right atrium. Using cardiopulmonary bypass with profound hypothermia and circulatory arrest, the inferior vena cava was opened below the renal veins, and the tumor was transected. That portion of the tumor above this transection was then extracted through a right atriotomy. Resection of the pelvic tumor was not thought to be feasible. The patient remains asymptomatic with stable pelvic tumor 1 year after the procedure.