Epithelioid hemangioendothelioma (EHE) is a rare tumor, first characterized by Sharon Weiss and Franz Enzinger in 1982[1] that both clinically and histologically is intermediate between angiosarcoma and hemangioma.

However, a distinct, disease-defining genetic alteration recently described for EHE indicates that it is an entirely separate entity from both angiosarcoma and hemangioma.

EHE was originally described as occurring most commonly in the veins of the extremities (arms and legs) and two organs, the liver[2] and lungs.

A balanced, reciprocal translocation t(1;3)(p36.3;q25) in EHE tumor cells was first described by Mendlick et al. in 2001.

[3] This led to the landmark paper by Tanas et al. in 2011[4] describing the specific genes involved in the translocation associated with the most common forms of EHE.

It binds and turns on a very important member of the TEAD family of transcription factors and this causes cells to proliferate.

Many upstream inputs regulate the Hippo signal which normally functions to turn off or de-activate TAZ by keeping it in the cytoplasm and out of the nucleus.

[13] In 2003 photographer and actress Kris Carr was diagnosed with a stable and low grade version of eHAE.