NCI Definition: A low-grade malignant blood vessel neoplasm. It is characterized by the presence of epithelioid endothelial cells. The neoplastic cells are arranged in cords and nests, which are embedded in a myxoid to hyalinized stroma. [1]

Epithelioid hemangioendotheliomas most frequently harbor alterations in WWTR1, CAMTA1, YAP1, TSC2, and TFE3 [2].

Most Commonly Altered Genes in Epithelioid Hemangioendothelioma

WWTR1-CAMTA1 Fusion, CAMTA1-WWTR1 Fusion, YAP1-TFE3 Fusion, YAP1 Fusion, and TFE3 Fusion are the most common alterations in epithelioid hemangioendothelioma [2].

Top Alterations in Epithelioid Hemangioendothelioma

Disease Details

Epithelioid Angiosarcoma, Epithelioid Angioendothelioma, Malignant epithelioid neoplasm, c/w epithelioid hemangioendothelioma
Malignant Vascular Neoplasm
OncoTree Name
Epithelioid Hemangioendothelioma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.