learn about EHe
EHE (epithelioid hemangio-endothelioma) is a rare vascular tumor that is often characterized as between benign and malignant. It has an unpredictable clinical course... it is often indolent for years punctuated by periods of tumor growth. Because it is so rare, most doctors have no knowledge of it and patients very often experience a circuitous route to reach a final diagnosis, including many misdiagnoses or misinformation. We strive to rectify this situation. Fortunately, there have been recent strides in the understanding of the disease and CRAVAT is dedicated to providing the latest information and resources to EHE patients and their doctors. We will provide access to current medical advances and literature relevant to EHE necessary for state-of-the-art understanding for this rare and unusual disease. We also continue to raise funds for relevant, basic research.
Why understanding EHE is important:
The underlying genetic cause of EHE results in persistent activation of TAZ, or its paralogue, YAP, genes that are now known to contribute to much more common cancers (e.g., breast, lung, pancreas,colon, etc). Learning to tame EHE would mean progress for all these other cancers. This makes EHE important as a model of pure, TAZ-driven cancer. Most cancers result from an accumulation of many genetic mutations, whereas only a single genetic change causes EHE. Thus EHE is also an exemplar of so-called precision medicine since treatment can be targeted specifically for the underlying TAZ-CAMTA mutation and this can be further calibrated for each patient based on their DNA breakpoint sequence. Activation of YAP/TAZ is also associated with, possibly even a requirment for metastasis. This means EHE is a very good model for the biology specific to the process of cancer cell metastasis. Interestingly, while metastasis is often a harbinger of bad outcome in other cancers, wide-spread disease is common in EHE but often doesn't in itself affect survival. That makes for a much 'cleaner' model of metastasis since this occurs in isolation from cinical decline. Therefore, beyond being a very interesting disease, EHE has value for every cancer patient insofar as understanding it will lead to better treatments of EHE as well as other more common cancers.
WHY THERE IS REAL REASON FOR OPTIMISM
It is important to convey to EHE patients and their families and especially the 'newbies' the several factors that explain why there is real reason for hope. Most important is that we have a first-rate scientist focused on finding a cure for EHE. Dr. Brian Rubin (shown below) is a professor of molecular pathology at Cleveland Clinic and was the first to identify the genetic alteration underlying the transformation of endothelial cells to EHE. He and his group more recently published a paper explaining how this occurs: how the fusion of two genes results in the cancer we know as EHE. They have developed a preliminary cell model of EHE and are now working to establish a genetically engineered mouse (GEM) model, too. These models allow us to understand the biology of the disease, a big step to finding a cure. Potential treatments can also be tested in the GEM. The next factor that has really changed the landscape for EHEers is the development of the EHE Facebook page. Here you can tap into the collective knowledge and support of a large number of people with interest and first-hand experience with EHE and all the related/linked interest groups. This is absolutely invaluable to the community. Finally, all of us together in different ways big and small have made contributions and raised money to support the efforts of Dr. Rubin's laboratory. He has been able to hire technical and scientific support and expand the efforts and scope of his scientific work. These three key items show that involvement from a grass roots level to the large medical center can unite to a achieve wonderful things. Lets keep going!!
PLEASE LINK HERE TO THE EHE REGISTRY AT PATIENT CROSSROADS.
Donating tissue (fresh or preserved) is one of the most important contributions to EHE research that patients can make. Click above to learn more. Dr. Rubin (in the video at right) shares his thoughts on how EHE patients can arrange to have fresh tissue (.e.g, after excision or biopsy) sent to him for analysis. Video courtesy of Jane Gutkovich, The EHE Foundation.
The Center for Research and Analysis of VAscular Tumors (CRAVAT) is an IRS approved 501(c)(3) not-for-profit organization that raises funds to increase awareness of and harness research to address rare vascular tumors. CRAVAT works with patients, clinicians and scientists to develop effective treatments for Epithelioid Hemangioendothelioma (EHE). Our mission statement: The central purpose of CRAVAT is to provide, on an international level, ongoing support for people with epithelioid hemangioendothelioma (EHE), and enable research that can give them better treatment for better outcomes.
Disclaimer: This information herein is for information only. The author has used all reasonable care and skill but makes no warranty as to the accuracy of any information herein, and cannot accept liability for any errors or omissions. The information provided is not intended nor implied to be a substitute for professional medical advice nor is it intended to be for medical diagnosis or treatment. If you are reading this, you have possibly been diagnosed already and are under medical instruction. DO NOT take the word of this brochure over that of your medical team.