Epithelioid Hemangioendothelioma (EHE)

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What is epithelioid hemangioendothelioma?

Epithelioid hemangioendothelioma, or EHE, is a rare cancer that grows from the cells that make up the blood vessels. This cancer can occur anywhere in the body with the most common sites being the liver, lungs, and bone. It usually happens in people between 30 and 50 years of age but can also occur in young children and older people.

How common is epithelioid hemangioendothelioma?

EHE is very rare, with only one in every one million people diagnosed with this cancer worldwide. Because EHE can be hard to diagnose, the actual number of people with EHE may be higher.

How is epithelioid hemangioendothelioma diagnosed?

People with EHE will have different symptoms depending on where the tumor is located. Common symptoms include:
•   Discomfort or pain in the abdomen
•   Nausea
•   Cough or trouble breathing
•   Fever
•   Pain and swelling around the tumor area
•   Weight loss
•   Problems moving or walking
•   Skin lumps and bumps that may appear red or blue
•   Broken bones
•   Coughing up blood

Imaging: If you have symptoms of EHE, your doctor will take images of the tumor using MRI, CT, X-ray, or PET scan to look at the size of the tumor and where it is in the body. They will also check for signs that the tumor has spread to other parts of the body.

Biopsy: To check if the tumor is EHE, your doctor will perform a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is.

How is epithelioid hemangioendothelioma treated?

Since EHE is so rare, there is no standard treatment. Treatment for each person is different and depends on where in the body EHE starts and if it has spread. You should see a doctor who is an expert in tumors that form in blood vessels to decide the best approach for your cancer.

Watch and wait: In some cases, the tumor grows very slowly, or even shrinks without any treatment. In this case it may be safest for your doctor to check your tumor often without treating it.

Surgery: Surgery is most often used to treat EHE that has not spread to other parts of the body. Surgery can also be used with chemotherapy and radiation therapy.

Radiation therapy: Doctors sometimes use radiation therapy to treat EHE, with or without surgery. Radiation therapy is most often used to treat EHE that grows in the bones. Because radiation therapy can cause other cancers in the future, it is important to discuss this option with your doctor.

Chemotherapy: Doctors may use chemotherapy with other treatments. It is important to discuss the side effects with your doctor.

Targeted therapy: Because EHE is made up of cells that form blood vessels, some doctors are trying treatments that target tumor blood vessels. These treatments are called anti-angiogenics.

Immunotherapy: Immunotherapy uses different treatments to help the body’s immune system kill tumor cells.

Embolization: In some cases, blocking the blood flow to the tumor, called embolization, can help shrink and kill the tumor.

Does epithelioid hemangioendothelioma run in families?

No, EHE is not passed down in families.

How does epithelioid hemangioendothelioma form?

We think that in EHE, chromosomes (the structures in your cells that contain all of your genes) break apart and get put back together in the wrong way. This can cause cells to not function like they should. In most EHEs, the WWTR gene that makes the TAZ protein joins with the CAMTA1 gene. In a small number of EHEs, the YAP gene joins with the TFE3 gene. TAZ and YAP work together to drive growth and scientists are looking at ways to block them. Doctors may look for these changes in chromosomes to confirm that the cancer is EHE.

What is the prognosis for someone with epithelioid hemangioendothelioma?

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:

Where the tumor is in your body
If the cancer has spread to other parts of your body
How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis.
Doctors estimate survival rates by how groups of people with EHE have done in the past. Given that there are so few EHE patients, survival rates may not be very accurate and may not consider newer treatments being developed.

The course of the disease for people with EHE can be hard to predict. Some people live a long time, even without treatment. In other cases, the cancer grows quickly and spreads, even with treatment. EHE that has spread to other parts of the body does not always lead to a poor prognosis. Some of this depends on where the EHE is located, so it is very important to discuss your case with you doctor to understand your prognosis.