Epithelioid hemangioendothelioma of the heart with distant metastases. A case report and literature review

Abstract

Epithelioid hemangioendothelioma is an uncommon intermediate-grade vascular tumor which most often presents in soft-tissue and is known to occasionally metastasize. A primary cardiac occurrence of this tumor was resected from the left atrium of a 71 year-old woman. Four months later, 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 identified as metastatic epithelioid hemangioendotheliomas. To the best of our knowledge, only one case of primary cardiac epithelioid hemangioendothelioma has been previously reported in the literature. We believe ours is the second such case and the first to demonstrate distant metastases. This rare case of primary cardiac epithelioid hemangioendothelioma with distant metastases is illustrative of many of the known features and behaviors of the tumor. The case is reviewed and discussed alongside a review of recent topical literature.