Epithelioid sarcoma | Sarcoma UK
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Epithelioid sarcoma

Epithelioid sarcoma is a very rare type of soft tissue sarcoma.

Epithelioid sarcoma is a very rare sarcoma that can occur anywhere throughout the body. It most often occurs in the hands, arms, feet and legs.

The cause of epithelioid sarcoma is unknown. But, researchers think that epithelioid sarcoma is caused by errors in a gene called SMARCB1. Researchers are still trying to find out why these errors happen and whether this causes epithelioid sarcoma.


There are an average of 19 cases of epithelioid sarcoma diagnosed every year in England.

Epithelioid sarcoma makes up just under 0.5% of all soft tissue sarcomas, and about 0.01% of all cancers.

For more information on these statistics, you can visit our soft tissue sarcoma data hub.

Signs and symptoms

Symptoms of epithelioid sarcoma can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with epithelioid sarcoma:

  • A lump or swelling within the soft tissue of the body under the skin, often in the hands, arms, feet and legs
  • This lump is usually hard but slow-growing and painless
  • These lumps often result in open sores called ulcers on the skin surface, which can be painful


There are two types of epithelioid sarcoma that you may be diagnosed with:

  • Distal – The most common type that commonly affects the hands, feet, legs and arms
  • Proximal – A less common type that mostly affects the central trunk, such as the chest, abdomen, pelvis and back

A specialist doctor will diagnose epithelioid sarcoma through a series of tests. These may include:

  • Physical examination – looking at and feeling any lump
  • A scan – taking pictures of the inside of the body using scans such as CT, PET or MRI scans
  • A biopsy – taking and testing a sample of the tumour. Using this biopsy, a doctor will look to see if the biopsy looks like epithelioid sarcoma and whether it contains the SMARCB1 error

To read more about these scans and tests, click here



  • The first treatment method for epithelioid sarcoma is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – this is known as “taking a margin”. This is to make sure that all of the cancer has been removed
  • Epithelioid sarcoma mostly affects the hands, arms, legs and feet, and  surgeons will do limb-sparing surgery so that the limb can continue to work well
  • Although very rare, if the cancer has spread throughout the limb, then the surgeons may have to perform a partial or full amputation to stop the cancer


  • This treatment uses high-energy radiation to destroy cancer cells
  • It can be used either before or after surgery
  • When used before surgery, radiotherapy aims to make the tumour smaller so that it can be more easily removed
  • When used after surgery, radiotherapy aims to kill off any remaining cancer cells that have not been removed by surgery


  • This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back or if it has spread to other parts of the body, also known as “metastasis”

Targeted and experimental therapies

  • In epithelioid sarcoma, researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”

Targeted therapy is a treatment that targets specific characteristics within cancer cells in order to stop them from growing and spreading.

Immunotherapy is a treatment that helps the person’s own immune system to fight and kill cancer cells.

  • There is a targeted therapy that can be used for treating epithelioid sarcoma. This is called tazemetostat and can be used if your tumour cannot be removed safely or if it has spread to other parts of the body.
  • You may be offered an opportunity to take part in a trial to investigate new diagnosis methods, drugs or treatments. Some studies also look at the care and well-being of patients. Your doctor or nurse can give you more information on opportunities for you to take part in a clinical trial.

Clinical Trials

For all sarcoma clinical trials based in the UK that are currently recruiting, you can check our Clinical Trials Hub.

After treatment

After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The follow-ups will usually include:

  • A chance to discuss symptoms
  • An examination to look for any signs of epithelioid sarcoma returning. This may include scans such as CT, PET or MRI scans
  • A chest x-ray to rule out any secondary cancers occurring in the lungs

Living with

Epithelioid sarcoma can reappear in the same area after the treatment of a previous tumour; this is called a “local recurrence”.

If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery and/or radiotherapy; your treatment will be assessed on an individual basis. It is useful to check for recurrences yourself through self-examination: your doctor or sarcoma clinical nurse specialist can tell you what to look for.

If you are worried about your cancer returning, please contact your doctor or nurse. They may decide to bring forward the date of your follow-up appointment to investigate your concerns.

What if my cancer spreads to another part of my body?

A recurrence of epithelioid sarcoma may be accompanied by cancer in other parts of the body. This is called “metastasis” or “secondary cancer”. Some people are diagnosed with sarcoma because their metastases have been discovered before their primary epithelioid sarcoma tumour. In epithelioid sarcoma patients, these secondary cancers may appear in the lungs, which is why a chest x-ray is taken at follow-up appointments.

Secondary cancers may also appear in the lymph nodes and scalp. Treatment for secondary cancer may involve surgery, radiotherapy or other therapy as appropriate; your treatment will be assessed on an individual basis.

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


More information –

A scientific review on the current management of epithelioid sarcoma

Last reviewed: 3 January 2023

Next review due: 3 January 2026


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