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

A 51-year-old woman presented with progressive right ventricular infundibul ar wall thickening and outflow obstruction. She had had an aorto-coronary b ypass 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 tumo r extended into the main pulmonary artery, but there was no other evidence of a primary or metastatic tumor. A biopsy specimen was obtained and based on the microscopic and immuno-histochemical findings (vimentin and Kp-1 pos itive) the diagnosis was primary cardiac malignant fibrous histiocytoma, wh ich 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 exten ded pulmonary artery obstruction.