FAQs

Where do I learn about EHE?

In addition to the information listed on The EHE Foundation website, we encourage you to join the EHE Facebook Group. The EHE community is your best resource for understanding this rare disease.

What is my prognosis?

EHE is currently quite unpredictable. It can be very slow growing, or not growing at all (in which case your prognosis is good)  but unfortunately also can be quite aggressive. The first question your doctor will have to figure out is if the tumors are growing and how fast.The prognosis is worse for rapidly progressing EHE. Symptoms, presence of pulmonary effusion for EHE of the lungs, EHE of the pleura and very abnormal blood work consider indications of worse prognosis as well. However, there is nothing written in stone! 

Is there cure for EHE?

If EHE is localized in soft tissue and removed before it spreads, some patients are considered actually cured and don’t have recurrence. If EHE is present in more than one location or present in the lungs or the liver, it unfortunately can’t be cured but with treatment, often can be controlled. 

What is the most effective treatment for EHE?

If EHE doesn’t progress, many doctors would recommend “wait and watch” approach while a patient undergoes routine monitoring (usually every 3 to 6 months). There is no standard treatment for progressing EHE. Different patients respond to different kind of drugs. Most of the time, doctors use drugs commonly used to treat other sarcomas.

Is there is a special diet recommended for EHE patients.

As with all other cancers, patients with EHE should try to eat healthy  and pay a special attention to foods that produce inflammation in our body, like red meat, too much sugars, saturated fats. Many of our patients are taking supplements that reduce inflammation, such as curcumin or green tea. You should always consult your doctor first.

Is there any research currently done on EHE?

Yes. Dr. Rubin from Cleveland Clinic assumed leadership in global EHE research. He identified a genetic malfunction that drives EHE and currently working on developing targeted therapy as well as many other aspects of comprehensive EHE research.

We currently bringing other researcher on board. One of the most distinguished scientist in immuno oncology Dr. Schreiber from Washington University in St. Louis agreed to lead an immunotherapy project on EHE. The EHE foundations of US, UK and Australia as well as EHE patients, their families and friends are currently supporting all research efforts.

Are there any clinical trials for EHE?

There are several clinical trials currently open to EHE patients.

1.Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That Is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery.

For the last several years Dr.Brian Rubin has been working on unlocking genetic driving mechanism behind EHE progression. His work resulted in identification of a pathway directly involved in supporting of EHE genetic malfunction .During the Lab experiments, Dr Rubin was able to disrupt this pathway with MEK inhibitor.

The clinical trial uses MEK inhibitor Mekinist. The purpose of the study to verify that what Dr.Rubin observed in his Lab actually translates in clinical effect in EHE patients. It is a multi institutional study which makes it very easy for enrolment in different parts of the country. You can see more information using following link.

https://clinicaltrials.gov/ct2/show/NCT03148275

2. Axitinib and Pembrolizumab in Subjects With Advanced Alveolar Soft Part Sarcoma and Other Soft Tissue Sarcomas

This is the only immunotherapy study currently listing EHE as designated sarcoma. Immunotherapy drug Pembrolizumab( Keytruda) is combined with anti angiogenic drug Axitinib.Both groups of drugs showed some effect in EHE patients. Their combination already presented synergetic effect in other cancers. Immunotherapy is gaining momentum and Dr. Breelyn Wilky from Sylvester Cancer Center in Miami is one of the leading immuno oncologists in sarcoma. She is a Primary Investigator in this trial. The study is conducted only in Miami. You can see more information using following link.

https://clinicaltrials.gov/ct2/show/NCT02636725

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