RESEARCH
Since our inception in 2010, CRAVAT has distributed more than $50,000 to scientists working to learn about and find treatments for EHE. Medical science has recently made great strides in understanding the causes and molecular biology underlying this rare sarcoma. This includes the hugely important work from the laboratory of Dr. Brian Rubin who identified the chromosomal abnormality that is present in virtually all EHE tumor cells. Obviously, the first step to treating a disease is understanging its cause. Dr. Rubin has made key contributions to this goal over the past two years and remains focused on finding a treatment for EHE. Our recipients to date include:
Dr. Brian Rubin, Professor of Molecular Pathology, Cleveland Clinic
Dr. Rubin characterized the specific chromosomal translocation that is now considered disease-defining for epithelioidhemangioendothelioma. Our grant will aid in the development of tissue culture (cell) and mouse (intact animal, or xenograft) models of EHE. These are not trivial undertakings but are necessary to test prospective treatments for efficacy in stopping growth of EHE tumors. We are at a critical point in this research where every financial contribution to this work can make a real difference in the rate of progress to identifying an effective treatment.
Liddy Shriver Sarcoma Initiative
CRAVAT has contributed to a grant funded by the Liddy Shriver Sarcoma Initiative. This collaborative effort will support work in Dr. Rubin’s laboratory intended to develop a diagnostic kit for EHE and to help develop experimental models of EHE to better understand the molecular biology of the disease and identify potential targets for treatment.
Dr. Douglas Feinstein, Research Professor at the University of Illinois at Chicago.
Dr.Feinstein is undertaking studies to test the theory that beta-blockers will reduce tumor burden in a mouse model of hemangioendothelioma.
Dr. Edward Breitschwerdt, Director, Vector Borne Disease Diagnostic Laboratory, North Carolina State University.
Dr. Breitschwerdt is studying the possible association of hemangioendothelioma and infection with species of the bacterium Bartonella.
MEDICAL LITERATURE
The best way to understand the current state of the art in cause, natural history and treatment of a disease is through the medical literature. A useful, standard search engine can be found at PubMed:
a few helpful papers
A recent search of ‘epithelioid hemangioendothelioma’ pulled 945 papers dating from 1982. I’ve listed a few helpful papers as a starting point for the uninitiated. I have not included case reports for they are essentially not useful in understanding patterns beyond the specific individual reported and are therefore not generalizable. Click below to be taken to some helpful papers.
The Medical Literature for almost every paper about EHE begins with a statement that reads something like -
“EHE is a vascular neoplasm intermediate between hemangioma (benign) and angiosarcoma”
Although the first part of this description is correct, the second part is not entirely accurate.
However
EHE is entirely distinct from both hemangioma and angiosarcoma.
We call EHE a vascular tumor
since it is caused by proliferation (unregulated growth) of endothelial cells – the cells that form the interior lining of blood vessels.
We know this now because
a genetic alteration responsible for EHE has recently been identified. It is entirely unique to EHE and is not seen in hemangiomas, angiosarcomas, or other vascular tumors.
The overall notion is right though:
EHE is neither benign like hemangioma nor is it usually aggressive like angiosarcoma.
We don’t recommend focusing too much on any one given paper that describes only one patient
Moreover,
most focus only on surgical approaches to treatment. This is partly because there are no standard non-surgical treatments for EHE and not enough EHE patients to run clinical trials that compare effectiveness of various treatments.
Many medical journal papers
about EHE describe only a single patient (“case reports”) and are therefore not very useful to you.
We don’t recommend focusing too much
on any one given paper that describes only one patient since such reports aren’t usually very helpful in evaluating or comparing treatments or understanding the range of outcomes in EHE. This takes what doctors call a randomized controlled clinical trial (RCT), or more simply, Clinical Trial.
However
there are two well-designed studies focused on liver transplant (generally very favorable results)
Unfortunately
there are currently no Clinical Trials comparing treatments for EHE.
Though neither has a control group (no transplant) for comparison
interestingly, outcomes among the transplanted patients are often favorable despite widespread disease. Therefore, the presence of tumors in other parts of the body is not usually considered a contraindication to transplant in EHE as it usually is in other forms of cancer.
The HEARD support group
Finally, the HEARD support group in collaboration with physicians at the University of Illinois at Chicago recently published the cumulative clinical experiences of roughly 200 patients with EHE in a recent issue of the journal Chest. They show that information from such an on-line registry can provide potentially helpful insights into the disease.