Ewing Sarcoma of the bone

Ewing sarcoma is a rare type of cancer that can develop in any bone. The most common bones affected are the:

  • pelvis
  • thighs (femur)
  • shins (tibia).

It is most commonly found in teenagers and young adults.

Pain is the most common symptom. The affected area may be swollen and tender to touch.

You may need specific tests and investigations to get a diagnosis. You will usually have an x-ray of the bone. Your doctors may remove a small piece of the tumour (a biopsy) which will be looked at under a microscope. This can show how quickly the cancer may grow. Other tests will check whether the cancer has spread elsewhere.

Most people with Ewing sarcoma will have a combination of treatments. Most people have chemotherapy to shrink the primary tumour and treat cancer cells that may have spread elsewhere. Then the tumour will be treated with surgery or radiotherapy 3 to 6 months later. Your surgeon will talk about different types of surgery with you before any decision is made. Chemotherapy will almost always continue after surgery or radiotherapy.

What is sarcoma?

Sarcomas are rare cancers that develop in the supporting tissues of the body. There are two main types: bone sarcomas and soft tissue sarcomas.

Bone sarcomas can develop in any of the bones of the body, but may also develop in the soft tissue near bones.

Soft tissue sarcomas can develop in muscle, fat, blood vessels, or any of the other tissues that support, surround and protect the organs of the body.

Ewing sarcoma most commonly occurs in the bone. This information should be read with our general information about bone cancer.


What is Ewing sarcoma?

Primary bone cancers are cancers that start in the bone. They are rare, with only about 580 new cases each year in the UK. There are several different types.

Ewing sarcoma is named after Dr James Ewing, who described the tumour in the 1920s. It is a primary bone cancer that can develop in any bone. The most common bones affected are the:

  • pelvis
  • thighs (femur)
  • shins (tibia).

It is most often found in teenagers and young adults, although it can happen at other ages. It is slightly more common in males than females.

Ewing sarcoma can also occur in the soft tissues instead of starting in the bone, although this is not common (this happens in about 1 in every 10 cases). This is called an extraosseous Ewing sarcoma. Sometimes these cancers are called primitive neuroectodermal tumours (PNET).

This information is about Ewing sarcoma of the bone. If you have one of the other types of Ewing sarcoma, our cancer support specialists can give you more information.


Causes of Ewing sarcoma

The exact causes of primary bone cancer are unknown. The development of Ewing sarcoma may be related in some way to rapid bone growth, which could explain why more cases of Ewing sarcoma are seen in teenagers. Research is taking place to find out more about what may cause Ewing sarcoma.


Signs and symptoms of Ewing sarcoma

Pain is the most common symptom of bone cancer. However, symptoms may vary depending on where in the body the cancer is and how big it is. There may be some swelling in the affected area and it may become tender to touch.

Primary bone cancer is sometimes found when a bone that has been weakened by cancer breaks after a minor fall or accident.

These symptoms can be caused by many things other than cancer. However, if you have persistent bone pain, especially if it occurs at night, you should get it checked by your doctor.


How Ewing sarcoma is diagnosed

Usually you begin by seeing your family doctor (GP), who will examine you and may arrange tests or x-rays. If your GP thinks you may have a Ewing sarcoma, they should refer you to a specialist hospital or bone tumour centre for further tests. Many of the tests for diagnosing bone tumours, such as bone biopsies, need to be done by an experienced team using specialist techniques.

The doctor at the hospital will ask you about your general health and any previous medical problems. They will examine you and look at the painful bone to check for any swelling or tenderness. You will probably have a blood test to check your general health.

You may need a variety of tests to diagnose Ewing sarcoma. An x-ray of the painful part of the bone will usually identify a tumour, although sometimes they can be hard to see.

A small piece of the tumour may be removed and looked at under a microscope (a biopsy). You may have other tests to check whether the cancer has spread to other parts of the body.

Bone x-ray

This uses x-rays to show the structure of the bone.

Biopsy

The doctor will take a sample of cells (a biopsy) to be checked by a pathologist (a doctor that specialises in cell types). If your doctor thinks you have bone cancer, the biopsy should be done at a specialist bone cancer centre.

Needle biopsy

A small sample of the tumour is taken from the affected bone using a needle. You will be given a local anaesthetic to numb the area. Sometimes a general anaesthetic is used.

Open or surgical 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. You may need this if a needle biopsy cannot be done, or if it does not give a clear result.

MRI (magnetic resonance imaging) scan

This test uses magnetism to build up cross-sectional pictures of your body. During the test, you'll 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's very noisy, but you will be given earplugs or headphones. You'll be able to hear, and speak to, the person operating the scanner.

If you have any metal implants (such as surgical clips or pacemakers), it will not be possible for you to have this test. In this situation, another type of scan may be used. 

CT (computerised tomography) scan

In some people with Ewing sarcoma, the cancer may spread to the lungs. You may have a CT scan to check for this. The scan takes a series of x-rays that build up a 3D (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 and cannot harm anyone you come into contact with.

You may be given a drink or an injection of dye, which allows certain 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 can normally go home as soon as the scan is over.

Bone scan

A bone scan is a more sensitive test than a 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’.

After you have the injection, you will need to wait 2 to 3 hours before you have the scan. You may want to take a magazine, book or MP3 player with you to help pass the time.

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. After having the injection, you will have to wait at least an hour before the scan. The scan usually takes 30 to 90 minutes. You can normally go straight home afterwards.

Usually, a scan that combines a CT scan and a PET (called PET/CT) is 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 sarcomas can sometimes spread to the bone marrow.

When Ewing sarcoma has been diagnosed or is suspected, a small sample of bone marrow is taken, usually from the hip bone (pelvis). It is then 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.

Waiting for test results can be an anxious time for you. It may help to talk about your worries with a relative or friend. You could also speak to one of our cancer support specialists.


Grading of bone sarcomas

Grading refers to the appearance of cancer cells under a microscope and gives an idea of how quickly a cancer may grow and develop. Low-grade means that the cancer cells look very much like normal cells. They are usually slow-growing and are less likely to spread. In high-grade tumours, the cells look very abnormal, are likely to grow more quickly, and are more likely to spread.

Ewing sarcomas are all considered to be high-grade tumours.


Staging of bone sarcomas

The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the type and the stage of the cancer helps the doctors plan the right treatment.

Most patients are grouped depending on if the cancer is found in only one part of the body (localised disease) or if it has spread from one part of the body to another (metastatic disease).

There are different staging systems used for bone cancer. One is the TNM staging system.

TNM stands for Tumour, Node and Metastases:

  • T describes the size of the tumour.
  • N describes whether the cancer has spread to lymph nodes.
  • M describes whether the cancer has spread to another part of the body (called metastatic or sec-ondary cancer).

This information is sometimes put together, along with the grade, to describe a number system:

Stage 1A

The cancer is low-grade and less than 8cm across.

Stage 1B

The cancer is low-grade and either bigger than 8cm across or in more than one place in the same bone.

Stage 2A

The cancer is high-grade and less than 8cm across.

Stage 2B

The cancer is high-grade and bigger than 8cm across.

Stage 3

The cancer is high-grade and is in more than one place in the bone where it started.

Stage 4A

The cancer is of any grade and has spread to the lung.

Stage 4B

The cancer is of any grade. It has spread to lymph nodes or other parts of the body other than the lungs.

If the cancer comes back after the first treatment, this is called recurrent or relapsed cancer.


Treatment for Ewing sarcoma

Ewing sarcomas are very rare, so they are usually treated at specialist hospitals by a team of doctors and other health care professionals. This means you may have to travel some distance for treatment.

A combination of treatments is used for Ewing sarcoma. This may include chemotherapy, surgery and radiotherapy. Treatment will depend on different things such as the position and size of the tumour.

Most people with Ewing 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 to 6 months later, the tumour will be treated either with surgery to remove the tumour, or with radiotherapy or a combination of both. 

Chemotherapy will almost always continue after surgery or radiotherapy.

Surgery

Major improvements have been made in surgery for bone cancer. In the past, the surgeon often needed to remove (amputate) the affected limb if Ewing sarcoma was found. Now they often just 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 called limb-sparing surgery.

Unfortunately, it is not always possible to use limb-sparing surgery, and sometimes 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 many different things. Your surgeon will talk about 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. On some wards, special counsellors may be available to talk about any worries you may have.

Chemotherapy

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. This is an important treatment for most people with Ewing 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.

You will then have further chemotherapy after surgery, to destroy any remaining cancer cells and stop the sarcoma from spreading outside the bone. This is called 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

Radiotherapy uses high-energy rays to destroy the cancer cells while doing as little harm as possible to normal cells. Radiotherapy works well in treating Ewing sarcoma, and may be used 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 instead 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 tell you what to expect.

Sometimes radiotherapy is given as proton therapy. You may need to go abroad to have proton therapy. Your doctor will be able to tell you more about this if it is relevant for you.

Possible late side effects

A small number of people may develop late side effects from the treatment they have had, sometimes many years after their treatment for Ewing sarcoma. These can include a change in the way the heart and kidneys 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 sarcoma

Research into new ways of treating Ewing 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 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 2012 trial is being carried out for children, young people and adults up to the age of 50 who have Ewing sarcoma. It is looking at different combinations of chemotherapy drugs. Your doctor or specialist nurse can tell you about any trials that may be relevant to your situation.

Before any trial can take place, it must have been approved by an ethics committee, which protects the interests of people taking part.

Your doctor or a research nurse will talk about 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 still receive the best standard treatment available.


Your feelings

You may have many different emotions, including anger, resentment, guilt, anxiety and fear. You may find yourself tearful, restless and unable to sleep. Or you may have feelings of hopelessness and depression. These are all normal reactions but it can be difficult and distressing to admit to them.

The need for support will vary from person to person and may depend on the treatment you receive and any side effects this causes. Your specialist will tell you about any potential side effects and how to deal with them before you begin any treatment.

Some hospitals have their own emotional support services with trained staff, and some of the nurses on the ward will have had training in counselling. You may feel more comfortable talking to a counsellor outside the hospital environment or to a member of your religious faith, if you are religious.

Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is available if you need it. Our cancer support specialists can give you information and support to help you cope.


Useful organisations

Cancer52 is an alliance of more than 50 organisations working to address the inequalities that exist in policy, services and research into the less common cancers and to improve outcomes for people with these highly challenging diseases.

Sarcoma UK and Bone Cancer Research Trust provides information and support to anyone affected by sarcoma, and aims to achieve the best possible standard of treatment and care for people with sarcoma.

Teenage Cancer Trust is a national charity providing specialist units, support, education and information for teenagers with cancer and their families.