Primary cardiac malignant fibrous histiocytoma in the right ventricular infundibulum treated with a cavo-pulmonary shunt and coronary embolization

Abstract

A 51-year-old woman presented with progressive right ventricular infundibular wall thickening and outflow obstruction. She had had an aorto-coronary bypass for left main coronary artery disease 1 year after radiation therapy for left mammary cancer. Enhanced computed tomography showed a mass in the right ventricular free wall with no connection to the mediastinum; the tumor extended into the main pulmonary artery, but there was no other evidence of a primary or metastatic tumor. A biopsy specimen was obained and based on the microscopic and immuno-histochemical findings (vimentin and Kp-1 positive) the diagnosis was primary cardiac malignant fibrous histiocytoma, which is very rare. A cavo-pulmonary artery connection lessened her symptoms, but embolization of the coronary artery to try and to reduce the mass had minimal effect. Four months after the tumor was diagnosed she died of extended pulmonary artery obstruction.