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(Circulation. 1998;97:703-704.)
© 1998 American Heart Association, Inc.

Images in Cardiovascular Medicine

Primary Malignant Fibrous Histiocytoma of the Heart Treated With Orthotopic Heart Transplantation

Henry A. Harlamert, MD; Jonathan S. Moulton, MD; ; William Lewis, MD

From the Department of Pathology and Laboratory Medicine (H.A.H., W.L.) and the Department of Radiological Services (J.S.M.), University of Cincinnati (Ohio) College of Medicine.

A24-year-old white man presented with dyspnea and fatigue. Physical examination revealed hepatosplenomegaly. A chest roentgenogram showed small pleural effusions without infiltrates. Ultrasound examination revealed ascites of the abdomen and pelvis and echogenic liver vessels that suggested congestive heart failure. A nuclear magnetic resonance image of the chest (Fig 1A) showed a 4x6-cm mass occupying the right ventricle. The mass filled the apex and obstructed the right ventricular cavity. A computerized tomogram (Fig 1B) localized the mass to the anterior and inferior right ventricular wall. For treatment, the tumor was debulked to reduce right ventricle obstruction. Histopathologically, the tumor was a malignant fibrous histocytoma. There was no evidence of an extra cardiac primary site or distant metastasis. The patient underwent subsequent cardiac transplantation.

View larger version (113K): [in this window] [in a new window]   Figure 1. A, Magnetic resonance T1W image (after contrast) of chest showing a right ventricular tumor almost completely occluding the outflow tract and a large right pleural effusion. B, Computerized tomogram with intravenous contrast enhancement at level of cardiac apex with tumor involving anterior right ventricular wall and interventricular septum. RA indicates right atrium; LV, left ventricle; P, pleural effusion; and T, tumor.

The explanted native heart contained the remaining tumor, measuring 6.2x3.5x8.5 cm, confined to the myocardium. The mass involved primarily the right ventricle and impinged significantly on the right ventricular cavity (Fig 2). It extended cephalad from the right ventricular apex, involved the tricuspid valve, and penetrated the right atrial wall. Anteriorly, it extended into the interventricular septum and involved the left ventricular . . . [Full Text of this Article]

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