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Epithelioid hemangioendothelioma (EHE) is a low-grade malignant vascular endothelial tumor. EHE of the spleen is rare and most often represents metastasis of EHE from other sites, or disseminated disease or multifocal disease. We report a case of EHE presenting as a splenic mass with multiple foci of bone lesion and mimicking primary myelofibrosis in the bone marrow biopsy.

Metastatic epithelioid hemangioendothelioma to the bone marrow with associated myelofibrosis resembling primary myelofibrosis