What are vascular sarcomas of the pleura?

Updated: Dec 25, 2019
  • Author: Joseph F Tomashefski, Jr, MD; more...
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The two major subtypes of primary vascular sarcomas of the pleura are epithelioid hemangioendothelioma (EHE) and angiosarcoma.

EHE, first described in the soft tissue by Weiss and Enzinger in 1982, is an angio-formative tumor of intermediate malignant potential composed of large neoplastic endothelial cells that have an epithelioid appearance and may be deceptively bland. [20] In addition to soft-tissue sites, EHE has been described in multiple other organs, including the lung, where EHE had originally been called "intravascular sclerosing bronchioloalveolar tumor (IVSBAT)." [3, 21]

Angiosarcoma, conversely, is considered an aggressive and highly malignant neoplasm that recapitulates the morphology of endothelial cells. Angiosarcomas may also arise in diverse locations, including the serosal membranes. Primary angiosarcoma of the serosal surfaces was described in detail by McCaughey et al in 1984. [22]

The morphological appearance of angiosarcoma is quite diverse and varies from well-differentiated slitlike vascular spaces to high-grade pleomorphic tumors that little resemblance to blood vessels. The morphological patterns of angiosarcoma also tend to vary with the site of origin. Epithelioid angiosarcoma, a frequent subtype among pleural angiosarcomas, is composed of nests of cohesive pleomorphic large cells that resemble carcinoma. [23]

The histological distinction between EHE and epithelioid angiosarcoma is not always well defined. Because of the rarity of these neoplasms, EHE and epithelioid angiosarcoma of the pleura are frequently grouped together in reported series of patients.

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