We report the case of a 76-year-old man with a malignant fibrous histiocytoma of the right ventricle and main pulmonary trunk, diagnosed through echocardiography and catheterization. Extensive resection of the right ventricular outflow tract, pulmonary valve apparatus, and main pulmonary trunk was performed, and the defect was repaired with a valveless ventriculo-pulmonary Dacron graft. The patient recovered uneventfully, and was doing well 18 months after surgery. To our knowledge, this is only the 15th case of primary malignant fibrous histiocytoma of the heart that has been documented in the literature since histologic criteria and cases were published in 1977-78, and the 2nd such case of a primary tumor that has arisen in a right cardiac chamber. The case is presented in detail, along with a review of the literature since 1978.
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