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Epithelioid Hemangioendothelioma (EHE/eHAE)

What is an epithelioid hemangioendothelioma?

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

How common is epithelioid hemangioendothelioma?

EHE is very rare, with only one in every one million people affected worldwide. Around 20 cases are diagnosed in the United States per year.

How is epithelioid hemangioendothelioma diagnosed?

People with EHE will have different symptoms depending on where the tumor is located. Common symptoms include:

  • 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 and trouble breathing

Imaging: If you have symptoms of EHE, your doctor will take images of the tumor using MRI, CT, X-ray, or PET scan to determine the size and location of the tumor. These factors determine which treatment options are available.

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

How is epithelioid hemangioendothelioma treated?

Given that EHE is so rare, there is no standard treatment. Treatment for EHE depends on where it starts and if it has spread, which is different for each patient. You should go to an expert in tumors that form in blood vessels to decide the best approach for your cancer. You can contact MyPART for help finding experts near you.

Surgery: Surgery is most often used to treat EHE. Surgery can also be combined with chemotherapy and radiation therapy.

Radiation therapy: Doctors sometime use radiation therapy to treat EHE, with or without surgery. Because radiation therapy can cause other cancers in the future, it is important to discuss this option with your doctor.

Chemotherapy: Doctors may combine 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: Some doctors use interferon to treat EHE. Interferon may block the growth of tumors directly, block the growth of tumor blood vessels, or turn on the immune system to fight cancer.

Does epithelioid hemangioendothelioma run in families?

No, EHE is not passed down in families.

How does epithelioid hemangioendothelioma form?

Scientists are always working to understand how cancer forms, but it can be hard to prove. We think that in EHE, chromosomes (the parts of 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 do 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. 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.

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