Epithelioid hemangioendothelioma of the liver with myocardial metastases.

Epithelioid hemangioendothelioma is an unusual type of endothelial-derived vascular tumor located mainly in the soft tissues of the limbs. The present report describes a 48-year-old woman with an epithelioid hemangioendothelioma located in the liver with very unusual metastases to the myocardium. While the disease itself was controlled by adriamycin the patient died as a consequence of portal hypertension due to the abundant fibrotic component distinctive of this type of tumor. An epithelioid appearance can sometimes lead to a mistaken diagnosis of metastatic carcinoma, as happened initially in our case. The endothelial origin of the tumor must be confirmed by electron microscope identification of Weibel-Palade bodies or by immunohistochemical staining of factor VIII-related antigen. To the authors knowledge, this is the first case of myocardial metastases from an epithelioid hemangioendothelioma of the liver to be reported in English in the medical literature.