This page explains epithelioid sarcoma, including symptoms, diagnosis, treatment, follow-up care and where to get support.
PRONUNCIATION
Epithelioid · ep-ih-thee-lee-oyd
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What is epithelioid sarcoma?
Epithelioid sarcoma is a very rare type of soft tissue sarcoma cancer.
Epithelioid sarcoma can happen anywhere in the body. It most often develops in the hands, fingers, arms, feet, or lower legs.
Who gets epithelioid sarcoma?
Epithelioid sarcoma can affect people of any age. It is most often diagnosed in teenagers and young adults, but it can also affect older adults.
Epithelioid sarcoma is more common in males than females.
On average, 19 cases of epithelioid sarcoma are diagnosed each year in England.
For more information on these statistics, you can visit our soft tissue sarcoma data hub.
What causes epithelioid sarcoma?
Doctors do not know exactly what causes epithelioid sarcoma.
Researchers have found that most epithelioid sarcoma tumours have changes in a gene called SMARCB1, or INI-1. This gene normally helps control how cells grow and divide.
Scientists are still trying to understand why these changes happen and how they lead to epithelioid sarcoma.
These gene changes happen in the tumour itself and are not usually passed down through families.
Symptoms of epithelioid sarcoma
The most common symptom is a lump in the soft tissue. The lump is often:
- painless
- firm to touch
- slow growing.
Some people notice more than one lump in the same area.
As the tumour grows, symptoms may include:
- changes to the skin near to the tumour, such as a sore or wound that does not heal
- swelling in the affected area
- pain or discomfort
- difficulty moving a nearby joint or limb.
Diagnosing epithelioid sarcoma
If you have symptoms that could be sarcoma, your GP will examine you and should refer you to a specialist team. The specialist team will use different scans and tests to see if you have epithelioid sarcoma.
You might have:
- an ultrasound scan – uses sound waves to create an image of an area of the body
- 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 area of the body
- a biopsy – involves taking and testing a sample of the tumour.
If tests show that you have epithelioid sarcoma, you may have more scans to help your specialist team understand the tumour and plan your treatment.
These may include:
- a CT scan of the chest to check your lungs
- scans of nearby lymph nodes (glands), as epithelioid sarcoma can sometimes spread to these areas.
We have more information about the different scans and tests you might have.
If you’re diagnosed with epithelioid sarcoma, 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.
Treating epithelioid sarcoma
Treatment depends on where the epithelioid sarcoma is, how big it is, and whether it has spread.
Your specialist team will work with you to plan the treatment that is right for you.
Surgery
Surgery is the main treatment for most people with epithelioid sarcoma.
Your surgeon will remove the tumour and a small area of healthy tissue around it. This is called taking a margin. This helps reduce the chance of the sarcoma coming back.
Epithelioid sarcoma often affects the hands, arms, feet, or legs. If this happens, your doctors will aim to keep the limb working as normally as possible. This is called limb-sparing surgery and is suitable for most people.
Very rarely, you might need an amputation if the tumour cannot be removed safely in another way. Your specialist team will explain all treatment options and support you through any decisions about surgery.
Because epithelioid sarcoma can sometimes spread to nearby lymph nodes (glands), your specialist team may also arrange scans or tests to check these areas.
Radiotherapy
Radiotherapy uses high-energy radiation to destroy cancer cells.
You may have radiotherapy:
- before surgery, to make the tumour smaller and easier to remove
- after surgery, to reduce the chance of the tumour coming back
- to help control symptoms, such as pain.
Chemotherapy
Chemotherapy uses medicines to destroy cancer cells.
Not everyone with epithelioid sarcoma will have chemotherapy. Your specialist team may recommend chemotherapy if:
- the cancer has spread
- the tumour is growing quickly
- the tumour cannot be removed with surgery.
Targeted therapy
Some people with epithelioid sarcoma may have a type of medicine called targeted therapy.
Targeted therapy works by targeting specific changes inside cancer cells to help stop them growing.
Some people may also be offered targeted therapy as part of a clinical trial.
Targeted therapy is not suitable for everyone with epithelioid sarcoma. Your specialist team will tell you if it is suitable for you and whether any clinical trials are available.
We have more information about clinical trials for sarcoma on Clinical Trials Hub.
After treatment
After your treatment for epithelioid sarcoma is finished, you will have follow-up appointments for several years. At these appointments, 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 uncertain or worried when treatment finishes. We have more information about living with and beyond sarcoma.
Recurrence
Epithelioid sarcoma can sometimes come back in the same area after treatment. This is called a local recurrence.
Sometimes epithelioid sarcoma spreads to other parts of the body. This is called metastatic or secondary cancer. The lungs are the most common place for epithelioid sarcoma to spread, but it can also spread to other areas.
If epithelioid sarcoma comes back, your specialist team will assess your situation and discuss the best treatment options with you. These may include surgery, radiotherapy, targeted treatment, or chemotherapy.
Your doctor or sarcoma clinical nurse specialist may show you how to check for signs that the tumour has come back.
It is normal to worry that sarcoma might return after treatment. Many people feel this way. If you are feeling anxious, speak to your healthcare team. They can answer your questions and help you find support.
We have more information about what happens if sarcoma comes back.
Living with epithelioid sarcoma
Living with epithelioid sarcoma can be hard. We offer information and support for your mental health, physical health and finances. No matter what you’re worried about, we’re here to support you.
If you have any more questions about epithelioid sarcoma, or if you need to talk to someone, our Support Line team are here for you.
More support
Contact the Sarcoma UK Support Line
Order our free soft tissue sarcoma booklet
Other cancer charities
Maggie’s
offers advice and support for adults and young people living with cancer.
Penny Brohn UK
Wellbeing support, online sessions and resources for people affected by cancer.
Shine Cancer Support
Support and community for people in their 20s, 30s and 40s affected by cancer.
Macmillan Cancer Support
Information about cancer diagnosis, treatment and life after treatment.
Trekstock
Community, exercise and wellbeing support for younger adults living with cancer.
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Last reviewed November 2026 | Next review due November 2029
All references are available upon request. Please email info@sarcoma.org.uk
