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Epithelioid Vascular Tumors

A Review

Shon, Wonwoo, DO*; Billings, Steven D., MD

Advances in Anatomic Pathology: May 2019 - Volume 26 - Issue 3 - p 186–197
doi: 10.1097/PAP.0000000000000228
Review Articles

Vascular tumors are a diagnostically challenging area. This is particularly true in the case of epithelioid vascular tumors. Not only is the distinction between different epithelioid vascular tumors challenging, but also the differential diagnosis may be substantially expanded by the inclusion of melanoma, carcinomas, and other epithelioid soft tissue tumors. Recently developed immunohistochemical markers and more comprehensive genetic characterizations continue to advance our understanding of epithelioid vascular tumors. The present paper briefly reviews and updates basic concepts with regard to the following epithelioid vascular tumors: epithelioid hemangioma, epithelioid angiomatous nodule, pseudomyogenic hemangioendothelioma, composite hemangioendothelioma, epithelioid hemangioendothelioma, and epithelioid angiosarcoma.

*Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, CA

Department of Pathology, Cleveland Clinic, Cleveland, OH

The authors have no funding or conflicts of interest to disclose.

All figures can be viewed online in color at

Reprints: Steven D. Billings, MD, Department of Pathology, Cleveland Clinic, 9500 Euclid Ave, L25, Cleveland, OH 44195 (e-mail:

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