Clinical recognition and management of cardiac metastatic disease: Observations in a unique case of alveolar soft-part sarcoma

A 22 year old woman presented with a three month history of progressive dyspnea, orthopnea and pedal edema. She had previously been in excellent health with the exception of surgery five years before for a tumor of her left thigh. The histopathologic diagnosis was alveolar soft-part sarcoma, a slow-growing granular cell sarcoma not previously known to metastasize to the heart. Her clinical course was characterized by increasingly severe dyspnea, edema, and ascites unresponsive to digitalis and diuretic therapy. Echocardiography during the most acute phase of the illness showed right ventricular dilitation, paradoxic septal motion and a normal left ventricle. Cardiac catheterization and angiography revealed a severely-obstructing filling defect moving with systole and diastole from the pulmonary trunk to the right ventricular outflow tract. At surgery, a mass of friable tumor tissue was removed from the right ventricular cavity, outflow tract, and pulmonary trunk with dramatic postoperative relief of symptoms and virtual abolition of the major hemodynamic abnormalities. Histopathologic examination showed the resected cardiac tumor to be alveolar soft-part sarcoma. To our knowledge, cardiac metastasis from alveolar soft-part sarcoma is thus far unique to the patient described in this report. Her unusual presentation and course, which extend the known clinical spectrum of this tumor, are discussed in the broader context of clinical recognition and management of cardiac metastatic disease.