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Epithelioid Hemangioendothelioma

Abstract

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.

Keywords

Fibrous Dysplasia Lytic Lesion Endothelial Differentiation Epithelioid Hemangioendothelioma High Mitotic Rate 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  1. 1.Laboratory of Orthopaedic PathologyBuenos AiresArgentina
  2. 2.Department of PathologyA.C. Camargo Cancer CenterSão PauloBrazil

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