EPITHELIOID HEMANGIOENDOTHELIOMA OF THE HEART WITH DISTANT METASTASES- A CASE-REPORT AND LITERATURE-REVIEW

Epithelioid hemangioendothelioma is an uncommon intermediate-grade vas cular tumor which most often presents in soft-tissue and is known to o ccasionally metastasize. A primary cardiac occurrence of this tumor wa s resected from the left atrium of a 71 year-old woman. four months la ter, she underwent gluteal exploration and resection of three nodules from her right buttock. These nodules, which resembled the previously resected tumor but showed features of malignant transformation, were i dentified as metastatic epithelioid hemangioendotheliomas. To the best of our knowledge, only one case of primary cardiac epithelioid hemang ioendothelioma has been previously reported in the literature. We beli eve ours is tile second such case and the first to demonstrate distant metastases. This rare case of primary cardiac epithelioid hemangioend othelioma with distant metastases is illustrative of many of the known features and behaviors of the tumor. The case is reviewed and discuss ed alongside a review of recent topical literature,