Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Angiosarcoma/Epithelioid Hemangioendothelioma, Pleural

  • Heather RuffEmail author
Reference work entry


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...

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References and Further Reading

  1. Lin, B., Colby, T., Gown, A. M., Hammer, S. P., Mertens, R. B., Churg, A., & Battifora, H. (1996). Malignant vascular tumors of the serous membranes mimicking mesothelioma: A report of 14 cases. The American Journal of Surgical Pathology, 20(12), 1431–1439.CrossRefPubMedGoogle Scholar
  2. Murali, R., Park, K., & Leslie, K. O. (2010). The pleura in health and disease. Seminars in Respiratory and Critical Care Medicine, 31(6), 649–673.CrossRefPubMedGoogle Scholar
  3. Ravi, V., & Patel, S. (2013). Vascular sarcomas. Current Oncology Reports, 15, 347–355.CrossRefPubMedGoogle Scholar
  4. Weissferdt, A., & Moran, C. A. (2010). Primary vascular tumors of the lungs: A review. Annals of Diagnostic Pathology, 14, 296–308.CrossRefPubMedGoogle Scholar
  5. Zhang, P. J., Livolsi, V. A., & Brooks, J. J. (2000). Malignant epithelioid vascular tumors of the pleura: Report of a series and literature review. Human Pathology, 31, 29–34.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.The University of Texas Medical BranchGalvestonUSA