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.
KeywordsLymphoma Sarcoma Myeloma Osteosarcoma Gadolinium
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