EHE is a rare vascular tumor that arises from the lining of blood vessels. It can appear almost anywhere in the body, but common sites include the liver, lungs, and bones.
The cause of EHE is presently unknown, and no proven treatments exist. EHE tumors can behave differently from one patient to the next, with some being stable for years while others progress quickly. The cancer often metastasizes throughout the body and possesses the ability to transform into an extremely aggressive state with little or no warning. In short, EHE is an unpredictable disease with potentially lethal consequences.
Since 2011, world-renowned pathologist Dr. Brian Rubin has been examining EHE on a molecular level at the Cleveland Clinic. His discovery of the specific gene fusion associated with EHE tumors led to the development of cell-based biological EHE models, a scientific breakthrough for EHE research and a quantum leap forward in the fight against epithelioid hemangioendothelioma.
Dr. Rubin's progress also introduced more expeditious diagnostic procedures as oncologists can now quickly identify the mutations associated with EHE. No longer are patients being incorrectly diagnosed, and while that is of course beneficial, it also means this cancer is becoming less rare.
There is no current treatment protocol for doctors to follow. When EHE is isolated to one organ, resections are usually recommended. If the cancer has spread but is indolent, a "wait and see" approach is common. Should the tumors grow and become more aggressive, a variety of therapies are utilized including anti angiogenic drugs, chemotherapy regimens as well as some interventional procedures. While these approaches have been successful in certain patients, to date no clinical research has scientifically justified any of these modalities.
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