Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Mediastinal Epithelioid Hemangioendothelioma

  • Annikka WeissferdtEmail author
  • Cesar A. Moran
Reference work entry


EH; Hemangioendothelioma; Histiocytoid hemangioma


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...

This is a preview of subscription content, log in to check access.

References and Further Reading

  1. Antonescu, C. R., Le Loarer, F., Mosquera, J. M., Sboner, A., Zhang, L., Chen, C. L., Chen, H. W., Pathan, N., Krausz, T., Dickson, B. C., Weinreb, I., Rubin, M. A., Hameed, M., & Fletcher, C. D. (2013). Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes, Chromosomes & Cancer, 52, 775–784.CrossRefGoogle Scholar
  2. Deyrup, A. T., Tighiouart, M., Montag, A. G., & Weiss, S. W. (2008). Epithelioid hemangioendothelioma of soft tissue: A proposal for risk stratification based on 49 cases. American Journal of Surgical Pathology, 32, 924–927.PubMedCrossRefGoogle Scholar
  3. Errani, C., Zhang, L., Sung, Y. S., Hajdu, M., Singer, S., Maki, R. G., Healey, J. H., & Antonescu, C. R. (2011). A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes, Chromosomes & Cancer, 50, 644–653.CrossRefGoogle Scholar
  4. Suster, S., Moran, C. A., & Koss, M. N. (1994). Epithelioid hemangioendothelioma of the anterior mediastinum. Clinicopathologic, immunohistochemical, and ultrastructural analysis of 12 cases. American Journal of Surgical Pathology, 18, 871–881.PubMedCrossRefGoogle Scholar
  5. Weiss, S. W., & Enzinger, F. M. (1982). Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer, 50, 970–981.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.MD Anderson Cancer CenterHoustonUSA