Rapidly progressing multiple cardiac rhabdomyosarcoma.

Abstract

A 47-year-old asymptomatic woman with heart murmur was referred to our hospital because of a left atrial tumor. No tumor had been found at previous echocardiography performed 6 months before. On examination, her vital signs were normal. Auscultation showed systolic regurgitant murmur and diastolic rumble at the cardiac apex. Electrocardiographical and chest radiographical findings were normal. Transthoracic and transesophageal echocardiography, and chest computed tomography (CT) revealed two cardiac tumors in the heart (Fig. 1). One tumor in the left atrium was attached to the free wall and the lateral portion of both anterior and posterior mitral leaflets (Fig. 1a–c), causing severe mitral regurgitation and stenosis of the mitral inflow (Fig. 1d). Another tumor in the left ventricle involved the anterior papillary muscle (Fig. 1a, b). No pericardial effusion was observed. Although we made a tentative diagnosis of cardiac myxomas, we suspected that the tumors were malignant because of the atypical features. Therefore, we recommended early resection. However, she refused the emergent operation because she had not experienced any symptoms. Thus, we planned to resect them 1 month after the visit. One month later, she was admitted to our hospital for a radical operation. Chest radiography on admission showed pulmonary congestion with bilateral pleural effusion and an enlarged cardiac silhouette. Repeated transthoracic echocardiography revealed enlargement of the tumors and large pericardial effusion (Fig. 2a, b). The mitral regurgitation and obstruction were aggravated by the tumors and restricted opening of the thickened mitral leaflets (Fig. 2c), which seemed to be caused by infiltration of the tumors. Mean pressure gradient through the obstruction was 15 mmHg. Moreover, a new tumor protruded into the pericardial space. The tumor seemed to arise from the left atrial appendage as if the tumor in the left atrium had invaded the left atrial wall (Fig. 2d). Thus, we emergently performed resection of the three tumors after removal of the bloody pericardial fluid and mitral valve replacement. The left atrial tumor of 7 9 5 9 5 cm arose from the free wall around the left atrial appendage and anterior commissure of the mitral valve. The mitral leaflets and the tip were thickened, suggesting infiltration of the tumors. The tumor was resected en bloc with the mitral leaflet. The left ventricular tumor of 3 9 2 9 2 cm involved the anterior papillary muscle. The second tumor was resected with the papillary muscle. The third tumor of 2 9 3 9 5 cm existed in the pericardial space and arose from the outer wall of the left atrium. We therefore resected this tumor with the left atrial wall and reconstructed the left atrial wall using equine pericardium. Y. Yokoi I. Yamadori Division of Clinical Laboratory, National Hospital Organization Okayama Medical Center, Okayama, Japan