UMMS Affiliation

Division of Cardiovascular Medicine, Department of Medicine; Department of Pathology; Department of Molecular, Cell, and Cancer Biology; Li-Weibo Institute for Rare Diseases Research; Graduate School of Biomedical Sciences

Publication Date


Document Type



Cardiovascular Diseases | Neoplasms | Pathology


Epithelioid hemangioendothelioma, first described in early 1980s,is a rare malignant vascular neoplasm with significant morbidity and mortality. Approximately 50% of epithelioid hemangioendotheliomas exhibit intravascular endothelial growth, yet their cellular origin, pathogenesis, and effective treatment remain undefined. Here, we identified stable nuclear expression of endothelial YAP1 (Yes1-associated transcriptional regulator) in pathological tissue samples from patients with epithelioid hemangioendothelioma. Mice expressing stable nuclear form of YAP1 in endothelial cells recapitulated the human intravascular epithelioid hemangioendothelioma phenotype. Sustained YAP1 activity induced mitosis and aberrant expression of lymphatic and epithelioid genes in blood endothelial cells. These results show sustained activation of endothelial YAP1 as a causal mechanism for intravascular epithelioid hemangioendothelioma.


heart neoplasms, morbidity, neoplasms, sarcoma, vascular diseases

Rights and Permissions

Copyright © 2021 The Authors. Arteriosclerosis, Thrombosis, and Vascular Biology is published on behalf of the American Heart Association, Inc., by Wolters Kluwer Health, Inc. This is an open access article under the terms of the Creative Commons Attribution Non-Commercial-NoDerivs License, which permits use, distribution, and reproduction in any medium, provided that the original work is properly cited, the use is noncommercial, and no modifications or adaptations are made.

DOI of Published Version



Jung R, Janardhan HP, Dresser K, Cotton JL, Hutchinson L, Mao J, Trivedi CM. Sustained Activation of Endothelial YAP1 Causes Epithelioid Hemangioendothelioma. Arterioscler Thromb Vasc Biol. 2021 Jul;41(7):2233-2235. doi: 10.1161/ATVBAHA.121.316300. Epub 2021 Jun 3. PMID: 34078092; PMCID: PMC8216595. Link to article on publisher's site

Journal/Book/Conference Title

Arteriosclerosis, thrombosis, and vascular biology

Related Resources

Link to Article in PubMed

PubMed ID


Creative Commons License

Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.