Angiosarcoma/Epithelioid Hemangioendothelioma, Pleural
Intravascular bronchioalveolar tumor; Lymphangiosarcoma
Epithelioid hemangioendothelioma (EHE) was originally called intravascular bronchioalveolar tumor when first described by Dail and Liebow in 1975, due to its epithelioid cytologic appearance and intra-alveolar growth pattern. However, following demonstration of its vascular nature, the term epithelioid hemangioendothelioma was coined by Weiss and Enzinger in 1982.
The term lymphangiosarcoma previously described tumors now classified simply as angiosarcomas; lymphangiosarcoma is no longer used as it is difficult to distinguish the endothelia of lymphatics from that of blood vessels and is of no known prognostic value.
Pleural angiosarcomas and pleural EHE are mesenchymal neoplasms of endothelial origin. Both tumors occur more commonly in extrapulmonary locations; however, these are two of the most common malignant vascular tumors described in the pulmonary system. When they occur in the pulmonary system, both...