Epithelioid hemangioendothelioma of the heart in association with myelodysplastic syndrome -: A case report and literature review

The case of a primary cardiac epithelioid heman-gioendothelioma (EHE) arising multifocally in the papillary muscle of the tricuspid valve and the deeper trabecular muscle in a 68-year-old male with myelodysplastic syndrome is presented. The tumor was an incidental autopsy finding. Histologically the tumor is characterized by proliferation of plump epithelial-like endothelial cells with the vascular differentiation being mostly expressed at a cellular level in the form of cytoplasmic vacuoles. In the differential diagnosis, metastatic carcinoma, cardiac myxoma as well as other epithelioid vascular neoplasms should be considered. EHEs are indolent tumors of intermediate malignancy with the potential to metastasize, even after a long time. Primary cardiac EHE is extremely rare. To our knowledge, only four EHEs of the heart have been reported in the literature. We believe, this is the first report of a cardiac EHE in this localization and the first one in association with myelodysplastic syndrome.