Epithelioid hemangioendothelioma (EHE)

Learn about epithelioid hemangioendothelioma (EHE). This rare cancer starts in cells that line blood vessels. It can form almost anywhere in the body.

Update Date: 02.09.2026

Overview
Symptoms
Causes
Risk factors
Complications
Prevention
Diagnosis
Treatment
Coping and support
Preparing for an appointment

Overview

An epithelioid hemangioendothelioma (EHE) is a rare cancer called a vascular sarcoma. It typically starts in cells called endothelial cells. These cells line blood vessels. They can form almost anywhere in the body. Common areas include the liver, lungs, soft tissue and bones.

EHE is part of the sarcoma family of cancers. It is not the same as an angiosarcoma, even though the two cancers can look alike under a microscope.

EHE can happen at any age. It most often is diagnosed in middle-aged adults. It is very rare in children.

EHE can act differently from one person to another. Some EHE tumors may grow slowly for years. Other EHE tumors may grow quickly. Treatment depends on where the EHE is located, whether it causes symptoms and whether it has spread to other parts of the body.

Symptoms

EHE does not always cause symptoms. Sometimes EHE tumors are found on scans done for another reason.

EHE symptoms can vary based on where the tumor is in the body. Symptoms in these areas may include:

  • Lungs: Cough, chest pain, or shortness of breath.
  • Liver: Large tumors may cause pain or discomfort in the belly area.
  • Soft tissues: A lump or pain.
  • Bones: Swelling, pain or broken bone.

When to see a doctor

Make an appointment with a healthcare professional if you have symptoms that worry you or are not going away.

Causes

The cause of EHE is not known. Researchers found certain gene changes in EHE tumor cells, but it’s not clear what causes these gene changes to happen.

Risk factors

The risk factors for EHE are not known.

Complications

EHE complications may include:

  • Tumor spread, also called metastasis, within an organ or to other parts of the body.
  • Fluid buildup around the lungs or in the belly.

Prevention

Researchers do not know what causes EHE. Factors that may increase a person's risk of developing EHE are not known.

Diagnosis

Epithelioid hemangioendothelioma (EHE) can be hard to diagnose because the tumors can look like other types of cancer on imaging tests or under a microscope.

Healthcare professionals often use a physical exam, imaging tests, a biopsy and lab tests to diagnose EHE.

Because EHE is rare, a pathologist with experience in EHE and sarcoma should review the tissue.

Physical exam

A member of your care team may ask about your symptoms and health history. A physical exam may help the care team learn more about your symptoms and look for other clues that help with your diagnosis.

Imaging tests

Imaging tests take pictures inside your body. The pictures can help your healthcare team understand the size and location of the EHE.

Your care team may use MRI, CT, PET or a combination of imaging tests to learn where tumors are in the body. Talk with your healthcare team about which imaging tests you may need.

  • Magnetic resonance imaging (MRI). MRI tests are used to study soft tissue, the liver or bones.
  • Computerized tomography (CT) scan. CT scans are used to study the lungs and see if the cancer spread to other organs.
  • Positron emission tomography (PET) scan. PET scans are used to see if cancer is affecting soft tissues and bones.

Biopsy

How a member of the care team collects the sample depends on where the tumor is in the body.

Types of biopsy procedures for EHE may include:

  • Image-guided core needle biopsy. This method uses imaging, such as an ultrasound or CT, to help a healthcare professional guide a needle through the skin and into a tumor to collect tissue samples.
  • Surgical biopsy. In some cases, your healthcare professional might suggest surgery to get a larger sample.

Treatment

There is no standard treatment for epithelioid hemangioendothelioma (EHE).

The type of treatment may depend on where the EHE is located, if there are symptoms and if it has spread to other parts of the body.

Types of treatment for EHE may include:

Active surveillance

During active surveillance, your care team checks the EHE with planned appointments, imaging tests and exams.

Surgery

Surgery may be an option for a tumor in an area where it can be removed. During surgery, a surgeon removes the tumor and some tissue around it. The goal is to remove the entire tumor, with no tumor cells at the edge of the removed tissue.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to specific points on your body.

Radiation therapy might be used instead of surgery if the tumor cannot be fully removed. It may be used before or after surgery to kill cancer cells.

Chemotherapy

Chemotherapy treats cancer with strong medicines. The medicines are often given through a vein. Chemotherapy may be an option if the EHE is growing quickly.

Targeted therapy

Targeted therapy uses medicines that target changes in cancer cells that help them grow. The medicines are often in pill form. By blocking these chemicals in the cancer cells, targeted treatments can cause cancer cells to die. Targeted medicines may be an option for EHE that cannot be removed, is causing symptoms or affects how an organ works.

Clinical trials

Researchers are studying new ways to treat EHE. Ask your healthcare professional whether a clinical trial might be an option for you.

Palliative care

Palliative care focuses on easing symptoms and supporting quality of life. It can be given along with EHE treatment.

Coping and support

An EHE diagnosis can stir up many feelings and questions. Your feelings may change from day to day. The following ideas may help.

  • Learn about EHE to make decisions about your care. If you'd like to know more about your cancer, ask your healthcare team for the details. Ask for good sources of information where you can learn more about your treatment options. Knowing more about your EHE and your options may help you feel more confident when making treatment decisions. Still, some people don't want to know the details of their cancer. If this is how you feel, let your care team know that.
  • Find a good listener. Talking with someone you trust may help as you manage an EHE diagnosis. This could be a friend, family member, counselor, medical social worker or faith leader.
  • Talk with other people who have EHE or sarcoma. You may find it helpful and encouraging to talk to others who have been diagnosed with EHE or other types of sarcoma. Ask your healthcare team about support groups in your area or contact the American Cancer Society or another cancer organization in your area to find resources. Find support online through Mayo Clinic Connect, a community where you can connect with others for support, practical information and answers to everyday questions.

Preparing for an appointment

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you or are not going away. If tests show you might have EHE, your healthcare team will likely refer you to a specialist.

Here's some information to help you get ready for your appointment.

What you can do to prepare

  • Write down your symptoms, including any that may seem unrelated to why you made the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medicines, vitamins or supplements that you're taking and the doses.
  • Keep all of your records that relate to your cancer diagnosis and treatment. Organize your records in a binder or folder that you can take to your appointments.
  • Consider bringing someone you trust. This person may help you remember what you discuss during the appointment.
  • Write down questions to ask your healthcare professional.

Questions to ask your doctor

Your time with your healthcare professional may be limited. Prepare a list of questions so that you can make the most of your time together. List your questions from most important to least important in case time runs out. Some basic questions to ask about EHE include:

  • What should I know about EHE?
  • Can you explain my pathology report to me? Can I have a copy for my records?
  • Do I need any more tests?
  • What treatment options are available for me?
  • What are the benefits and risks of each treatment you recommend?
  • What are the side effects of each treatment option?

Other questions could include:

  • How will each treatment option affect my daily life? Can I continue working?
  • Is there one treatment you recommend over the others?
  • How do you know that these treatments will benefit me?
  • What would you recommend to a friend or family member in my situation?
  • How quickly do I need to make a decision about cancer treatment?
  • What happens if I don't want cancer treatment?
  • What websites or books about my cancer do you recommend?
  • Are there any clinical trials or newer treatments that I should consider?

You also can ask other questions during your appointment.

What to expect from your doctor

Be prepared to answer some questions about your symptoms and your health, such as:

  • When did you first begin experiencing symptoms?
  • Are your symptoms always present, or do they come and go?
  • How bad are your symptoms?
  • What makes your symptoms better?
  • What makes your symptoms worse?

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