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

Abstract

A 24-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 4ϫ6-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 6.2ϫ3.5ϫ8.5 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. 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.

Primary Cardiac and Pericardial Neoplasms: Radiologic-Pathologic Correlation

“…Radiologic Features.-The most common radiographic abnormality in patients with cardiac sarcoma is cardiomegaly. Other findings include heart failure, pleural effusion, focal cardiac mass, pulmonary consolidation, and pericardial effusion (2,7,91,(94)(95)(96). Because of their location, the radiographic findings of left atrial sarcomas can mimic those of left atrial myxoma.…”

“…Even after complete tumor excision, however, local recurrence and metastatic disease occur frequently and early, usually within 1 year (2,90,94). Heart transplantation has been performed in some patients with unresectable cardiac sarcoma with satisfactory results (92,95,100). Chemotherapy and radiation therapy have not proved beneficial for the treatment of affected patients (92).…”

Malignant Fibrous Histiocytoma of the Heart: Case Report and Review of 46 Cases in the Literature.

“…A second operation was not indicated in our patient because of the tumor dissemination into the chest wall. It has been reported that heart transplantation offers good results for cardiac malignancies (45,57), however the indication is considered to be strictly limited due to the highly metastatic potential. In summary,we presented a rare case of cardiac MFH and reviewed 46 additional cases in order to clarify the nature of this rare disease.…”