This information is about a rare type of sarcoma of the bone called Ewing’s sarcoma. It 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 people diagnosed each year in the UK. There are several different types of bone cancer.

This type of sarcoma is named after Dr James Ewing, who described the tumour in the 1920s.

It is a cancer which can develop anywhere in the body, although it most often starts in the bone (a primary bone tumour). Any bone can be affected, but the pelvis, thigh bone (femur) and shin bone (tibia) are the most common places. Ewing’s sarcoma is most commonly found in teenagers and young adults, and is slightly more common in males than females.

Although Ewing’s sarcoma is a type of bone cancer, Ewing’s sarcoma can also very rarely occur in the soft tissues rather than starting in bone. This is called an extraosseous Ewing’s sarcoma. Sometimes these cancers are called primitive neuroectodermal tumours (PNET).

This information refers to Ewing’s sarcoma of the bone. If you have one of the other types of Ewing’s sarcoma, our nurses can give you further information.

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 sarcomas.

Pain is the most common symptom of bone cancer. However, symptoms may vary depending on the position in the body and the size of the cancer. 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 the person has had a minor fall or accident.

The symptoms described above can be caused by things other than cancer. However any persistent bone pain, especially if it occurs at night, should be checked by your doctor.

Usually you begin by seeing your family doctor (GP), who will examine you and may arrange any necessary tests or x-rays. Your GP will probably refer you to a specialist surgeon (called an orthopaedic surgeon) who can carry out tests to decide if you need a referral 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 done to check your general health.

Bone x-ray This involves the use of x-rays to show the structure of the bone.

MRI (magnetic resonance imaging) scan This test is similar to a CT scan, but uses magnetism instead of x-rays to build up cross-sectional pictures of your body. During the test you will be asked to lie very still on a couch inside a large metal cylinder that is open at both ends. The whole test may take up to an hour. It can be slightly uncomfortable and some people feel a bit claustrophobic during the scan. It is very noisy, but you will be given earplugs or headphones to wear. A two-way intercom allows you to talk with the people controlling the scanner. If you have any metal implants (such as surgical clips, pacemakers, metal in the eye from previous accidents or trauma) it will not be possible for you to have this test.

Needle biopsy For this test a small sample of the tumour is taken from the affected bone. The doctor looks at the sample under a microscope to identify the exact type of tumour that you may have.

Open biopsy This type of 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 to do this if a needle biopsy can’t be done or doesn’t give a clear diagnosis.

Bone scan This is a more sensitive test than the simple x-ray and shows up any abnormal areas of bone more clearly. For this test, 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’.

CT (computerised tomography) scan A CT scan takes a series of x-rays that builds up a three-dimensional picture of the inside of the body. The scan is painless and takes from 10 to 30 minutes. CT scans use a small amount of radiation, which is very unlikely to harm you and will not harm anyone you come into contact with. You will 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 that allows particular areas to be seen more clearly. For a few minutes this may make you feel hot all over. 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.

Most people are able to go home as soon as their scan is over.

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 if 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.

Chest x-ray In Ewing’s sarcoma the cancer may spread to the lungs. A chest x-ray is usually done to check for this.

Ewing’s sarcoma is treated by a combination of treatments which may include chemotherapy, surgery and radiotherapy. Treatment will depend on individual factors such as the position and the size of the tumour.

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 that you may have to travel some distance to have your treatment.

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 three and six months later, the tumour will be treated with either surgery to remove the tumour, or radiotherapy. Chemotherapy will often continue after either surgery or radiotherapy.

Surgery

Major improvements in surgery for bone cancer have been made. In the past, it was often necessary to remove (amputate) the affected limb if Ewing’s sarcoma was found. Now, however, it is 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 is 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 is often helpful to talk to someone who has had the same operation that you are going to have. The medical and nursing staff will be able to arrange this for you. On some wards a special counsellor may be available to discuss any worries you may have.

Chemotherapy

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 is often given before surgery and may shrink large tumours enough to avoid amputation. The course of chemotherapy will then 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 sometimes have unpleasant side effects. Any side effects that do occur can often be controlled well with medicine.

Radiotherapy

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 often very effective in treating Ewing’s sarcoma and this treatment is often used after chemotherapy and 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 general side effects, such as feeling sick (nausea) and tiredness. 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 will be able to advise you what to expect.

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 different countries working together and so they may take some time to complete for results of the trials to be helpful.

Clinical trials for Ewing’s sarcoma are especially important so that more people can be cured. Currently the Euro-Ewing 99 trial is being carried out. Your doctor or specialist nurse can discuss with you 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 must 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 withdraw from a trial at any stage and will then receive the best standard treatment available.

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 times, let your doctor know as soon as possible.

The need for practical and emotional support will vary from person to person and may depend on the treatment you receive and any side effects the treatment may cause. Your specialist will inform you of any potential side effects and how to deal with them before you agree to treatment.

Many different emotions may affect you. Anger, guilt, anxiety and fear are some of the most common feelings that people have. You may find yourself tearful, restless and unable to sleep. You may have feelings of hopelessness and depression. These are all normal reactions, but it is often difficult and distressing to admit to them.

You do not have to cope with these feelings on your own and there are people available to help you. You may also find it helpful to talk to your family and friends about how you feel. Some hospitals have their own emotional support services with specially-trained staff, and some of the nurses on the ward will have received training in counselling. Some people feel more comfortable talking to a counsellor outside the hospital environment or to a religious leader.

Our cancer support service can put you in touch with counselling services in your area or see our list of counselling organisations.

This section has been compiled using information from a number of reliable sources, including:

• Oxford Textbook of Oncology (2nd edition). Eds. Souhami et al. Oxford University Press, 2002.
• Textbook of Uncommon Cancers (3rd edition). Eds. Raghavan et al. Wiley Publishers, 2006.
• Pathology and Genetics of Tumours of Soft Tissue and Bone – World Health Organisation Classification of Tumours. Fletcher et al. Oxford University Press, 2002.

For further references, please see general bibliography.