Epithelioid haemangioendothelioma

This page explains epithelioid haemangioendothelioma (EHE). It includes information about diagnosis and treatment, as well as where to get more support if you’re diagnosed with EHE. 

Epithelioid haemangioendothelioma is pronounced ‘ep-ih-thee-lee-oyd hee-man-jee-oh-en-doh-thee-lee-oh-muh’.

If you have any questions about EHE or if you need to talk to someone, our Support Line team are here for you.

What is epithelioid haemangioendothelioma?

Epithelioid haemangioendothelioma (EHE) is a very rare type of sarcoma cancer. It starts in the cells that line blood vessels.

EHE can happen anywhere in the body. It is most common in the:

• lungs
• liver
• bones
• soft tissue, such as muscle or skin.

EHE behaves differently in different people. In some people, it grows very slowly and may stay stable for a long time. In others, it grows more quickly or spreads to other parts of the body. Some people are diagnosed with more than one tumour at the same time, or with tumours in more than one part of the body. This is called multifocal EHE.

Because EHE affects everyone differently, your specialist team will plan treatment around your individual situation.

Who gets epithelioid haemangioendothelioma?

EHE is most commonly diagnosed in adults aged 30 to 50. It is slightly more common in females than males.

Around 14 people are diagnosed with EHE every year in England.

What causes epithelioid haemangioendothelioma?

Researchers think EHE may happen when changes occur in a cell’s DNA. DNA carries the instructions that tell cells how to work. Researchers are still trying to understand why these changes happen and how they lead to EHE. These changes happen by chance and are not passed down through families.

Symptoms of epithelioid haemangioendothelioma

The symptoms of EHE depend on the size of the tumour and where it is in your body5. Some people have no symptoms at all. This can happen when the tumour is small or grows very slowly.

If symptoms do develop, they usually depend on the part of your body affected.

If EHE is in the lungs

Symptoms may include:

• a cough that does not go away
• shortness of breath
• chest pain
• coughing up blood.

If EHE is in the liver

Symptoms may include:

• pain in your tummy
• feeling sick
• tiredness
• weight loss that you cannot explain
• high temperature (a fever).

If EHE is in the bones

Symptoms may include:

• bone pain, particularly at night
• a lump or swelling
• bones that break more easily.

If EHE is in soft tissue or skin

Symptoms may include:

• a lump that is getting bigger or changing
• changes in skin colour or texture.

Because EHE develops from blood vessel cells, lumps or skin changes can sometimes look:

• darker than the surrounding skin
• like a bruise.

Diagnosing epithelioid haemangioendothelioma

If you have symptoms that could be sarcoma cancer, your GP will examine you and may refer you to a specialist team. The specialist team will use different scans and tests6 to see if you have EHE.

You might have:

• an MRI scan – uses magnets to create an image of the tissues of the body.
• a CT scan – takes a number of X-rays to make a 3D image of an affected area.
• a PET scan – uses a small amount of radiation to scan the inside of your body. It can sometimes be used alongside a CT scan or an MRI scan.
• a biopsy – taking and testing a sample of the tumour.

We have more information about the different scans and tests you might have.

Some people do not have any symptoms. Doctors sometimes find EHE by chance during scans or tests for another reason.

If you’re diagnosed with EHE, a team of experts called a multidisciplinary team (MDT) at a sarcoma specialist centre will review your results and work with you to plan your treatment.

After treatment  

After your treatment for EHE is finished, you will have follow-up appointments for several years. This is where your doctor checks for signs of sarcoma coming back.

During follow-up care, you will have:

• a chance to discuss symptoms
• examinations to check for any signs of sarcoma returning
• imaging scans such as a CT or MRI
• chest X-rays to check for cancer spreading to the lungs
• a chance to talk about how you’re feeling and any worries you have.

We know that some people can feel lost when treatment finishes, and that is completely normal. We have more information about living with and beyond sarcoma.

Recurrence

EHE can sometimes come back in the same area after the treatment of a previous tumour. This is called a local recurrence.

Sometimes EHE can spread to other parts of the body. This is called metastasis or secondary cancer. EHE most often spreads to the lungs, liver, or bones. Because of this, you may have chest X-rays or other scans as part of your follow-up appointments. These help your specialist team check for changes in the lungs and other areas of the body.

If EHE comes back, you may be offered more treatment, such as chemotherapy, surgery, and radiotherapy. Your treatment will be assessed on an individual basis.

Your doctor or sarcoma clinical nurse specialist may show you how to check for signs of recurrence.

It’s understandable to feel worried that sarcoma might come back after treatment. Many people feel this way. If you’re feeling anxious, speak to your healthcare team. They can answer your questions and help you find support.

We have more information about if sarcoma comes back.