Mediastinal Epithelioid Hemangioendothelioma

Synonyms

EH; Hemangioendothelioma; Histiocytoid hemangioma

Definition

The term “epithelioid hemangioendothelioma” (EH) was first used by Weiss and Enzinger in 1982 to describe an unusual tumor of the soft tissues with an indolent clinical course. Its striking epithelioid appearance, vascular origin, and presumed clinical course intermediate between hemangioma and angiosarcoma led to the proposal of its name. The most common primary site of these tumors is the soft tissue of the extremities, but they are also known to occur in many other locations such as the bone, liver, lung, or skin. Primary mediastinal EH are rare tumors that arise from the mediastinal soft tissue or from the great vessels. Epithelioid hemangioendotheliomas not uncommonly present as multifocal disease or with multiorgan involvement prompting careful radiological investigations to search for additional involved sites (Suster et al. 1994).

Epithelioid hemangioendotheliomas belong to a spectrum of epithelioid vascular...