Epithelioid hemangioendothelioma is a rare, low- to intermediate-grade malignant neoplasm, characterized by small cords or nests of epithelioid cells with endothelial differentiation set on a myxohyaline or chondroid background. Bones of the extremities respond for at least half the cases. More than half of osseous tumors are multifocal. Pain and swelling are usually present. Intracytoplasmic vacuoles sometimes containing erythrocytic material are characteristic (“blister cells”). Marked cellular atypia, high mitotic rate, extensive cell spindling, and necrosis usually correlate to more aggressive tumors. WWTR1-CAMTA1 gene fusion is considered specific for EHE. Wide en bloc surgical resection is the preferred choice of treatment.
KeywordsFibrous Dysplasia Lytic Lesion Endothelial Differentiation Epithelioid Hemangioendothelioma High Mitotic Rate
- Antonescu CR, Le Loarer F, Mosquera JM, Sboner A, Zhang L, Chen CL, Chen HW, Pathan N, Krausz T, Dickson BC, Weinreb I, Rubin MA, Hameed M, Fletcher CD. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosomes Cancer. 2013;52(8):775–84.CrossRefPubMedCentralPubMedGoogle Scholar
- Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumors of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013.Google Scholar