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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 free wall and apex. The endocardium overlying the tumor was generally intact, with a few foci of adherent fibrin. Grossly, the tumor was white-to-tan and gelatinous, with a rubbery consistency (Fig 3). Microscopically, sections showed a myxoid appearance with variable cellularity (Fig 4). Mitoses were abundant, and abnormal mitoses were seen frequently. Tumor cells were oval and fusiform and had indistinct cell borders and abundant cytoplasm. Immunohistochemical stains of the tumor showed reactivity with antibodies to smooth muscle actin but not with antibodies to S-100 protein, cytokeratin, or desmin.

View larger version (112K): [in this window] [in a new window]   Figure 2. Gross photograph of tumor involving right ventricle, tricuspid valve, and right atrium with severe compression of right ventricular cavity.

View larger version (137K): [in this window] [in a new window]   Figure 3. Gross photograph of cut section of tumor showing white-to-tan, gelatinous appearance.

View larger version (127K): [in this window] [in a new window]   Figure 4. Microscopic section showing pleomorphic, fusiform tumor cells. One large cell contains an atypical mitotic figure (inset). Bar=50 µm.

Primary cardiac sarcomas are rare. The patient is doing well 4 months after surgery.

Footnotes

Reprint requests to William Lewis, MD, College of Medicine, University of Cincinnati, 231 Bethesda Ave, Cincinnati, OH 45267-0529.

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Harlamert, H. A. Articles by Lewis, W. Search for Related Content PubMed PubMed Citation Articles by Harlamert, H. A. Articles by Lewis, W.