Cardiac angiomyolipoma: radiologic and pathologic correlation

Primary tumors of the heart are rare. We report a case of large cardiac angiomyolipoma (hamartoma) that presented as a fat-containing tumor mass on imaging studies, diagnosed radiographically as teratoma. The patient was admitted through the emergency room at Tainan Municipal Hospital because of severe dyspnea. A chest radiograph revealed marked widening of the mediastinum. Echocardiography and computed tomographic scanning of the thorax showed a mass of mixed density with calcification. A teratoma with intrapericardial invasion was suspected. Sternotomy disclosed a large intrapericardial lobulated mass (34 x 30 x 12 cm, 3,150 g) arising from the right atrium, with severe adhesion to the origin of the inferior vena cava. Histopathologic examination demonstrated an angiomyolipoma of the heart. To our knowledge, this is the largest cardiac angiomyolipoma reported. We report this case to emphasize that a differential diagnosis of angiomyolipoma must be included in a patient with a fat-containing cardiac tumor.