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Epithelioid hemangioendothelioma
Epithelioid hemangioendothelioma (eHAE) is a rare tumor, first characterized by Sharon Weiss and Franz Enzinger that both clinically and histologically is intermediate between angiosarcoma and hemangioma. Some now refer to it as low grade epithelioid angiosarcoma. It typically occurs in the 20 - 40 age range with no sex predilection, although the overall age range involved is much broader. It has an indolent course, some have survived for decades with multi-organ disease.〔James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. ISBN 0-7216-2921-0.〕 Before their description the tumor had been reported under a variety of other names, including histiocytoid hemangioendothelioma, intravascular bronchoalveolar tumor (in the lung), and sclerosing cholangiocarcinoma (in the liver). In the lung and liver, common sites of metastatic tumor, it was most likely to be confused with carcinoma (tumor with epithelial differentiation), a far more common type of tumor.
It is a vascular cancer in the lining (intima) of blood vessels, originally described most commonly in the veins of the extremities (arms and legs) and two organs, the liver and lungs. It has since been described in organs throughout the body. In addition to liver and lungs, bones and skin have been the most frequent organs.
==Genetics==
The cytogenetics of eHAE are not well characterized, as yet: t(1;3)(p36.3;q25) and a small supernumerary marker chromosome involving 22qll have been described in some patients.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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