Primary Malignant Fibrous Histiocytoma of the Heart Treated With Orthotopic Heart Transplantation
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 4×6-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.
The explanted native heart contained the remaining tumor, measuring 6.2×3.5×8.5 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.
Primary cardiac sarcomas are rare. The patient is doing well 4 months after surgery.
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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.
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