Abstract
Epithelioid hemangioendothelioma is a relatively uncommon lesion usually presenting in soft tissues. The occurrence in the mediastinum is exceptional rare. Histologically, this tumor is characterized by epithelioid cells with intracytoplasmic vacuoles in a hyalinized or mucinous stroma. Occasionally, spindle cells or osteoclast-like giant cells can be observed. Herein, we present a case of epithelioid hemangioendothelioma in a 38 year-old Chinese male. The tumor was predominantly composed of abundant spindle cells with marked atypia and scattered osteoclast-like giant cells reminiscent of malignant fibrous histiocytoma. The unusual histological appearance can pose a great diagnostic challenge. It may be easily misdiagnosed, especially if the specimen is limited or from fine-needle aspiration.Virtual slideshttp://www.diagnosticpathology.diagnomx.eu/vs/5804918529726307
Highlights
Epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial tumor first described by Weiss and Enzinger in 1982 [1]
Tissue blocks were cut into 4-μm slides, deparaffinized in xylene, rehydrated with graded alcohols, and immunostained with the following antibodies: cytokeratin (CK), CD68, Vimentin, CD31, CD34, CEA, Desmin, thyroid transcription factor 1 (TTF-1), CDX-2, CD5, Calretinin, Actin(SM), CD117, S-100 and Ki67
Scattered multinucleated osteoclastlike giant cells were present within the background of the diffuse spindle cells
Summary
Epithelioid hemangioendothelioma (EHE) is a rare vascular endothelial tumor first described by Weiss and Enzinger in 1982 [1]. It is usually considered an intermediate vascular neoplasm between a benign hemangioma and a highly aggressive angiosarcoma [2]. EHE can occur at any age, but usually in the adulthood. It can occur in soft tissues and various organs including lung, bone, liver and skin [3,4,5]. EHE is histologically characterized by cords and nests epithelioid cells with intracytoplasmic vacuoles in a myxohyaline stroma. The tumor may be present with spindle cells and
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