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. 
Epithelioid hemangioendotheliomas most frequently harbor alterations in WWTR1, CAMTA1, YAP1, TSC2, and TFE3 .
WWTR1-CAMTA1 Fusion, CAMTA1-WWTR1 Fusion, YAP1-TFE3 Fusion, YAP1 Fusion, and TFE3 Fusion are the most common alterations in epithelioid hemangioendothelioma .
There are 3 clinical trials for epithelioid hemangioendothelioma, of which 3 are open and 0 are completed or closed. Of the trials that contain epithelioid hemangioendothelioma as an inclusion criterion, 3 are phase 2 (3 open).
Eribulin, lenvatinib, and pembrolizumab are the most common interventions in epithelioid hemangioendothelioma clinical trials.
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.