Ewing sarcoma

What is Ewing sarcoma?

Ewing sarcoma is a type of primary bone cancer (also called bone sarcoma). Sarcomas are rare cancers that develop in the supporting tissues of the body. Supporting tissues include bone, cartilage, tendons, fat and muscle.

There are 2 main types of sarcoma:

bone sarcomas (also called primary bone cancer)
soft tissue sarcoma.

Primary bone cancer can develop in any bone in the body. It can also develop in the soft tissue near bones.

The bones most commonly affected by Ewing sarcoma are:

pelvis
thigh (femur)
shin (tibia)
ribs and shoulder blades.

Ewing sarcoma is most often found in teenagers and young adults, but it can happen at other ages. It is slightly more common in men than women.

Sometimes Ewing sarcoma can start in the soft tissue near the bone. This is called extraosseous Ewing sarcoma. Extra means outside, osseous means bone. It can also be called soft tissue Ewing sarcoma. Sometimes these cancers are called primitive neuroectodermal tumours (PNETs).

These are often treated in the same way as Ewing sarcoma that starts in the bone.

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What is cancer?

Symptoms of Ewing sarcoma

Pain is the most common symptom of bone cancer, including Ewing sarcoma. But symptoms may vary, depending on what part of the body the cancer is in.

The area may be:

swollen
painful when touched.

Primary bone cancer is sometimes found when a bone breaks after a minor fall or accident. This is because it has been weakened by the cancer.

Most of the time, these symptoms are caused by other conditions that are more common than bone cancer. But if you have any swelling or unexplained bone pain, get it checked by your GP.

Tell your GP if the pain happens at night and when you are resting.

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Do I have cancer?

Causes of Ewing sarcoma

The exact causes of primary bone cancer are not known. Research is happening to find out more about what may cause Ewing sarcoma.

Diagnosis of Ewing sarcoma

You usually start by visiting your GP. They will check the area, and your general health. They may arrange some tests or x-rays.

Your GP will refer you to a specialist sarcoma centre for tests if they:

are not sure what the problem is
think you may have a bone cancer.

You may have some of the following tests to diagnose Ewing sarcoma:

Bone x-ray

You may have an x-ray to check an area of bone for cancer. An x-ray shows a picture of the bone. This can give information about where the cancer may have started or what type of bone cancer it is.
MRI scan

An MRI scan uses magnetism to build up a detailed picture of areas of your body.
Bone biopsy

A bone biopsy means the doctor takes a sample of tissue from the bone to be checked for cancer under the microscope.
Bone marrow sample

The bone marrow is the spongy material inside the bones where blood cells are made. When Ewing sarcoma is suspected or diagnosed, the doctor will take a small sample of bone marrow. This is because Ewing sarcoma can sometimes spread to the bone marrow. They usually take the sample from 1 side of the hip bone (pelvis). Sometimes they also take a sample from the other side.
CT scan

A CT scan takes a series of x-rays, which build up a three-dimensional (3D) picture of the inside of the body. You may have a CT scan of the affected bone and also the chest.
PET-CT scan

This is a combination of a CT scan, which takes a series of x-rays to build up a three-dimensional (3D) picture, and a positron emission tomography (PET) scan.

Bone biopsies and other tests to diagnose bone cancer are done by sarcoma specialists. Tests may be done on the tissue sample to find out if there are any genetic changes in the sarcoma cells. This is sometimes called cytogenetic testing. The tissue sample is also tested to look for any proteins the cancer cell might be producing.

This information can help tell your doctor the type of sarcoma you have and if certain treatments are suitable for you.

We have more information about diagnosing bone cancer.

Waiting for test results can be a difficult time. We have more information that can help.

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Staging and grading

The results of your tests give your cancer doctor information about the grade and the stage of the cancer.

The stage of the cancer refers to the size of the cancer and whether it has spread outside the bone.

The grade of the cancer is how the cancer cells look under the microscope. This gives doctors an idea of how quickly a cancer may grow and develop.

The most common grading system for primary bone cancer uses the following 3 grades:

Grade 1 - cancer cells are low-grade and look like normal bone cells. They are usually slow-growing and less likely to spread.
Grade 2 - cancer cells are high-grade and look abnormal. The cells are likely to grow more quickly and are more likely to spread.
Grade 3 - cancer cells are high-grade and look more abnormal than grade 2. This means the cancer is more likely to come back (recur) or spread to other parts of the body.

All Ewing sarcomas are high-grade.

We have more information about staging and grading bone cancer.

Treatment for Ewing sarcoma

Ewing sarcoma is rare, so it is treated by a team of doctors and other healthcare professionals at a hospital with a specialist sarcoma treatment centre.

Your test results are discussed by a team of specialist health care professionals. If your tests show a diagnosis of bone cancer, a team of specialist doctors and other professionals called a multidisciplinary team (MDT) will meet. They will discuss the best possible treatment for you.

After the MDT meeting, your cancer doctor or specialist nurse will explain the treatment options and possible side effects to you. They will also talk to you about things to consider when making a decision.

The treatment you have depends on a number of things, including:

the position of the cancer
the size of the cancer
if it has spread (its stage)
the grade of the cancer
your general health.

The main treatments for Ewing sarcoma are surgery, radiotherapy and chemotherapy. You usually have a combination of these treatments:

Surgery

If possible, surgery is used to remove Ewing sarcoma. Surgery aims to remove the affected area of bone. Some people might have reconstructive surgery at the same time as the operation. If you have a bone sarcoma in an arm or leg, it is usually possible to remove the sarcoma with limb-sparing surgery. Very rarely, the best treatment is to remove the whole of the affected limb (amputation).
Chemotherapy

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. It is often given before surgery to shrink the tumour and improve the results of the operation. You usually have more chemotherapy after surgery, to destroy any remaining cancer cells. Sometimes it is used to shrink large cancers to avoid having to remove a limb (amputation). We have more information about the drugs that may be used to treat Ewing sarcoma.
Radiotherapy

Radiotherapy uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. Ewing sarcoma responds very well to radiotherapy. It may be used with surgery, either before or after an operation. Radiotherapy may be given with chemotherapy when surgery is not possible. This is called chemoradiation. Radiotherapy may also be used to treat Ewing sarcoma that has spread to the lung. Sometimes a type of radiotherapy called proton beam therapy (PBT) is used.

You may be offered some treatments as part of a clinical trial.

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After Ewing sarcoma treatment

Follow up

After you finish treatment, you will have regular check-ups for a few years. This will include chest x-rays. You may also have scans and blood tests.

If you have any problems, or notice any new symptoms in between your regular follow-up appointments, tell your cancer doctor as soon as possible.

Possible late effects

A small number of people have side effects that continue after treatment. This will vary depending on the size and position of the tumour and the treatment. You may have rehabilitation to help you recover and manage any side effects.

Sometimes side effects develop after treatment and some may happen many years later. Your cancer doctor or specialist nurse will explain more about any possible late effects and what can help to manage them. Always tell them if side effects are not improving.

Sex and fertility

Cancer and its treatment can sometimes affect your sex life. There are ways to help your sexual well-being and to manage any problems.

Cancer and its treatment may cause changes to your body that affect your body image. There are things that can help you to cope with these changes.

Treatment for sarcoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:

Well-being and recovery

Even if you already have a healthy lifestyle, you may choose to make some positive lifestyle changes after treatment.

Making small changes such as eating well and keeping active can improve your health and well-being and help your body recover.

Getting support

After finishing treatment, you may still be coping with difficult feelings. Talking to your family and friends or health professionals about how you feel can help to support your well-being.

Organisations such as Sarcoma UK and the Bone Cancer Research Trust can provide information and support. Cancer52 works to improve the quality of life for people with rare cancers.

Macmillan is also here to support you. If you would like to talk, you can:

call the Macmillan Support Line for free on 0808 808 00 00
chat to our specialists online
visit our bone cancer forum to talk with people who have been affected by bone cancer, share your experience, and ask an expert your questions.

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Finishing cancer treatment

About our information

References

Below is a sample of the sources used in our Ewing sarcoma information. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

European Society for Medical Oncology ESMO. Clinical Practice Guideline for diagnosis, treatment and follow-up. S.J. Strauss et al. December 2021. Available from Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up - Annals of Oncology (accessed May 2023)

Journal of Clinical Medicine. Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives. Stefan K. Zollner et al. April 2021. Available from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8071040/ (accessed May 2023)

National Institute for Health and Care Excellence (NICE). Bone and soft tissue sarcoma - recognition and referral: Diagnosis of bone and soft tissue sarcoma. Last revised August 2020. Available from https://cks.nice.org.uk/topics/bone-soft-tissue-sarcoma-recognition-referral/diagnosis/ (accessed May 2023)

Journal of Pathology and Translational Medicine. What’s new in soft tissue and bone pathology 2022–updates from the WHO classification 5th edition. Erica Y. Kao 1 and Jose G. Mantilla 2. November 2022. Available from What’s new in soft tissue and bone pathology 2022–updates from the WHO classification 5th edition - PMC (nih.gov) (accessed May 2023)

Reviewers

This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by senior medical editor Fiona Cowie, Consultant Clinical Oncologist.

Our cancer information has been awarded the PIF TICK. Created by the Patient Information Forum, this quality mark shows we meet PIF’s 10 criteria for trustworthy health information.

The language we use

We want everyone affected by cancer to feel our information is written for them.

We want our information to be as clear as possible. To do this, we try to:

use plain English
explain medical words
use short sentences
use illustrations to explain text
structure the information clearly
make sure important points are clear.

We use gender-inclusive language and talk to our readers as ‘you’ so that everyone feels included. Where clinically necessary we use the terms ‘men’ and ‘women’ or ‘male’ and ‘female’. For example, we do so when talking about parts of the body or mentioning statistics or research about who is affected.

You can read more about how we produce our information here.