Ewing's sarcoma of the bone
Ewing’s sarcoma is a rare type of sarcoma of the bone, and this information should ideally be read with our information about primary bone cancer.
Primary bone cancers are cancers that start in the bone. They are rare, with only about 400 new cases each year in the UK. There are several different types.
Ewing's sarcoma is named after Dr James Ewing, who described the tumour in the 1920s. It is a cancer that can develop anywhere in the body, although it most often starts in the bone (called a primary bone tumour). Any bone can be affected, but the pelvis, thigh bone (femur) and shin bone (tibia) are the most common sites.
This sarcoma is most commonly found in teenagers and yound adults, although it can happen at other ages. It’s slightly more common in males than females.
Ewing’s sarcoma can also occur in the soft tissues rather than starting in the bone, although this isn’t common. This is called an extraosseous Ewing’s sarcoma. Sometimes these cancers are called primitive neuroectodermal tumours (PNET).
This information is about Ewing’s sarcoma of the bone. If you have one of the other types of Ewing’s sarcoma, our cancer support specialists can give you more information.
Causes of Ewing’s sarcoma
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The exact causes of primary bone cancer are unknown. The development of Ewing’s sarcoma may be related in some way to rapid bone growth, which could explain why more cases of Ewing’s sarcoma are seen in teenagers. Research is taking place to find out more about what may cause Ewing’s sarcoma.
Signs and symptoms of Ewing's sarcoma
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Pain is the most common symptom of bone cancer. However, symptoms may vary depending on the position of the cancer in the body and its size. There may be some swelling in the affected area and it may become tender to touch.
Bone cancer is sometimes discovered when a bone that has been weakened by cancer breaks after a minor fall or accident.
The symptoms described above can be caused by many things other than cancer. However, any persistent bone pain should be checked by your doctor, especially if it occurs at night.
How Ewing's sarcoma is diagnosed
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Your family doctor (GP) will examine you and may arrange tests or x-rays. If a Ewing's sarcoma is suspected, you may be referred directly to a specialist hospital or bone tumour centre for further investigations. Many of the specific tests for diagnosing bone tumours, such as biopsies, need to be done by an experienced surgeon using specialist techniques.
The doctor at the hospital will take your full medical history before doing a physical examination. This will include an examination of the painful bone to check for any swelling or tenderness. You will probably have a blood test to check your general health.
A variety of tests and investigations may be needed to diagnose Ewing's sarcoma. An x-ray of the painful part of the bone will usually identify a tumour, although sometimes they can be difficult to see.
A small piece of the tumour will be removed and looked at under a microscope (called a biopsy). Other tests are done to check whether the cancer has spread elsewhere. These may include a bone x-ray, chest x-ray, bone scan, bone marrow sample, further biopsies and an MRI or CT scan.
This involves the use of x rays to show the structure of the bone.
A small sample of the tumour is taken from the affected bone. This may be done under a general anaesthetic. The doctor looks at the sample under a microscope to identify the exact type of tumour you may have.
An open biopsy is not often used, as the needle biopsy is much quicker and simpler. In an open biopsy a small piece of bone is removed during a minor operation while you are under a general anaesthetic. It may be necessary if a needle biopsy can’t be done, or if it doesn’t give a clear diagnosis.
MRI (magnetic resonance imaging) scan
An MRI scan uses magnetism to build up a detailed picture of areas of your body. Before the scan you may be asked to complete and sign a checklist. This is to make sure it’s safe for you to have an MRI scan. Before having the scan, you’ll be asked to remove any metal belongings including jewellery.
During the test you'll be asked to lie very still on a couch inside a long cylinder. The whole test may take up to an hour. It is painless but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It’s also noisy, but you will be given earplugs or headphones. You'll be able to hear, and speak to, the person operating the scanner.
CT (computerised tomography) scan
A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless and takes just a few minutes. CT scans use small amounts of radiation, which are very unlikely to harm you or anyone you come into contact with. You'll be asked not to eat or drink for at least four hours before the scan.
You may be given a drink or an injection of dye, which allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. If you are allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it is important to let your doctor know beforehand.
You will probably be able to go home as soon as the scan is over.
A bone scan is a more sensitive test than the bone x-ray, and shows up any abnormal areas of bone more clearly. A small amount of a mildly radioactive substance is injected into a vein, usually in your arm. Abnormal bone absorbs more radioactivity than normal bone, so these areas are highlighted and picked up by the scanner as hot spots.
PET (positron emission tomography) scan
A PET scan uses low-dose radioactive sugar to measure the activity of cells in different parts of the body. A very small amount of a mildly radioactive substance is injected into a vein, usually in your arm. A scan is then taken a couple of hours later. Areas of cancer are usually more active than surrounding tissue, so they show up on the scan.
Sometimes a type of scan that combines a CT scan and a PET scan may be used, as it can give more information about the position and size of a tumour.
Bone marrow sample
The bone marrow is the spongy material inside the bones where our blood cells are made. Ewing’s sarcomas can sometimes spread to the bone marrow.
When Ewing’s sarcoma has been diagnosed or is suspected, a small sample of bone marrow is taken from the hip bone (pelvis) and looked at under a microscope to see whether it contains any abnormal cells. The bone marrow sample may be taken under a local anaesthetic, but in younger children it is usually done under a general anaesthetic.
Treatment for Ewing's sarcoma
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As Ewing’s sarcomas are very rare, they are usually treated at specialist hospitals by a team of doctors and other health professionals. This means you may have to travel some distance for treatment.
A combination of treatments is used for Ewing's sarcoma. This may include chemotherapy, surgery and radiotherapy. Treatment will depend on individual factors such as the position and size of the tumour.
Most people with Ewing’s sarcoma will receive chemotherapy to shrink the primary tumour and also treat any cancer cells that may have spread to other parts of the body. Between 3-6 months later, the tumour will be treated either with surgery to remove the tumour, or with radiotherapy.
Chemotherapy will almost always continue after surgery or radiotherapy.
Major improvements have been made in surgery for bone cancer. In the past, it was often necessary to remove (amputate) the affected limb if Ewing's sarcoma was found. Now it's often possible just to remove the affected part of the bone and some of the healthy tissue around it. The bone is then replaced with a specially designed metal replacement (prosthesis), or a bone graft (bone taken from another part of the body). If the cancer affects a bone in or near a joint, the whole joint can often be replaced with an artificial one. These operations are known as limb-sparing surgery.
Unfortunately, it's not always possible to use limb-sparing surgery, and occasionally an amputation may be the only way to treat the cancer. This is often the case when the cancer has spread from the bone into the nerves and blood vessels around it.
The type of surgery you have will depend on a number of factors. Your surgeon will discuss the different types of surgery with you in detail before any decision is made about your treatment.
It's often helpful to talk to someone who has had the same operation that you’re going to have. The medical and nursing staff will be able to arrange this. On some wards, special counsellors may be available to discuss any worries you may have.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. This is an important treatment for most people with Ewing’s sarcoma, as it can greatly improve the results of surgical treatment. It's often given before surgery and may shrink large tumours enough to avoid amputation.
The course of chemotherapy will continue after surgery, to destroy any remaining cancer cells and stop the sarcoma from spreading outside the bone. This is known as adjuvant chemotherapy.
Chemotherapy can make you feel better by relieving the symptoms of the cancer, but it can have unpleasant side effects. Any side effects that do occur can often be controlled with medicines.
Radiotherapy treats cancer by using high-energy rays to destroy the cancer cells while doing as little harm as possible to normal cells. Radiotherapy is very effective in treating Ewing’s sarcoma, and may be used together with chemotherapy, either before or after surgery. In some cases, surgery is not possible - for example, if the tumour is in the spine. In these cases radiotherapy may be given in place of surgery.
Radiotherapy can cause side effects such as redness of the skin (erythema) and tiredness (fatigue). These side effects can be mild or more troublesome, depending on the strength of the radiotherapy dose and the length of your treatment. The radiotherapist can advise you what to expect.
Possible late side effects
A small number of people may develop late side effects, sometimes many years after their treatment for Ewing’s sarcoma. These can include a change in the way the heart and lungs work and a slight increase in the risk of developing another cancer in later life.
Your doctor or nurse will explain more about any possible late side effects.
Clinical trials for Ewing's sarcoma
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Research into new ways of treating Ewing’s sarcoma is going on all the time. Cancer doctors use clinical trials to assess new treatments. As bone cancers are very rare, clinical trials are usually organised by cancer specialists from several countries working together, and they may take some time to complete.
Clinical trials for Ewing’s sarcoma are especially important for learning more about such a rare cancer, in order to cure more people. Currently a large trial called the Euro-Ewing 99 trial is being carried out for children, young people and adults up to the age of 50 who have Ewing's sarcoma. It is looking at different combinations of chemotherapy drugs with surgery and radiotherapy. Your doctor or specialist nurse can discuss any trials that may be relevant to your situation.
Before any trial is allowed to take place, it must have been approved by an ethics committee, which protects the interests of patients taking part.
Your doctor or a research nurse will discuss the treatment with you, so that you have full understanding of the trial and what it means to take part. You may decide not to take part or to withdraw from a trial at any stage. You will then receive the best standard treatment available.
Follow-up for Ewing's sarcoma
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After your treatment is completed, you will have regular check-ups and x-rays. These will continue for several years. If you have any problems, or notice any new symptoms in between these check-ups, let your doctor know as soon as possible.
The need for practical and emotional support varies from person to person and may depend on the treatment you receive and any side effects it causes. Your specialist will tell you about any potential side effects, and how to deal with them, before you agree to treatment.
You may experience many different emotions. Anger, guilt, anxiety and fear are some of the most common feelings people have. You may be tearful, restless and unable to sleep. You may have feelings of hopelessness and depression. These are all normal reactions, but it can be difficult and distressing to admit you have them.
Many people find it helpful to talk things over with their doctor or nurse or with one of our cancer support specialists. Family members and close friends can also offer support.
This information has been compiled using a number of reliable sources, including:
DC Harmon et al. Treatment of the Ewing’s sarcoma family of tumours. UpToDate (accessed November 2012).
Raghavan, et al. Textbook of Uncommon Cancers. 3rd edition. Wiley. 2006.
TF Delaney et al. Clinical presentation, staging and prognostic factors of the Ewing’s family of tumours. UpToDate (accessed November 2012).
The ESMO/European Sarcoma Network Working Group. Bone sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology. 2012. Volume 23: Issue Supplement 7.
Tobias J, Hochhauser D. Cancer and its Management. 6th edition. Wiley-Blackwell. 2010.
With thanks to Dr Beatrice Seddon, Consultant Clinical Oncologist, and the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network.