Ewing's sarcoma in children
Ewing’s sarcoma is a type of bone cancer in children. This information describes Ewing’s sarcoma, its symptoms, diagnosis and possible treatments.
More children than ever are surviving childhood cancer. There are now new and better drugs and treatments. But it remains devastating to hear that your child has cancer, and at times it can feel overwhelming. There are many healthcare professionals and support organisations to help you through this difficult time.
Understanding more about the cancer your child has and the treatments that may be used can often help parents to cope. We hope you find the information here helpful. Your child's specialist will give you more detailed information. If you have any questions it’s important to ask the specialist doctor or nurse who knows your child’s individual situation.
You may also find it helpful to read this information alongside our section on children’s cancers, which contains more information about cancers in children, their diagnosis and treatment, and the support services available.
Sarcomas are rare types of cancer 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 skeleton, 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's sarcoma in children
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Ewing’s sarcoma is the second most common primary sarcoma in children and young people. This type of cancer is a bone cancer and most commonly occurs in the long bones, ribs, pelvis and spine (vertebral column).
Fewer than 30 children in the UK develop Ewing’s sarcoma each year. It usually occurs in the teenage years, and is more common in boys.
Although Ewing's sarcoma is a type of bone cancer, it can also occur very rarely in the soft tissues. This is called extraosseous Ewing's sarcoma. Another type of Ewing’s sarcoma is a primitive neuroectodermal tumour (PNET). These can be found in either the bone or soft tissue.
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 times of rapid bone growth, which may explain why more cases are seen in teenagers. Like other cancers, it’s not infectious and cannot be passed on to other people.
Signs and symptoms of Ewing's sarcoma in children
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Pain is the most common symptom of bone cancer. However, symptoms may vary depending on the position and size of the cancer. There may be some swelling in the affected area if the tumour is close to the surface of the body and it may become tender to touch. Bone cancer is sometimes discovered when a bone that has been weakened by cancer breaks after the child has a minor fall or accident.
How Ewing's sarcoma is diagnosed
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Usually you begin by seeing your GP, who will examine your child and may arrange tests or x rays. If a sarcoma is suspected, your GP should refer your child directly to a specialist hospital or bone tumour centre. A variety of tests and investigations are needed to diagnose Ewing’s sarcoma, including an x-ray of the painful part of the bone, a chest x-ray and a blood test. A specialist doctor will remove a small piece of the tumour to look at under a microscope (biopsy). Other tests such as a PET scan, a bone marrow biopsy, and an MRI or CT scan may also be done.
Any tests and investigations that your child needs will be explained to you.
Treatment for Ewing's sarcoma in children
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A combination of various treatments is used to treat Ewing's sarcoma. These include chemotherapy, surgery and radiotherapy. Treatment will depend on a number of factors, including the size and position of the tumour.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells.
This is an important treatment for most children with Ewing’s sarcoma and has greatly improved the results of surgical treatment. A number of different chemotherapy drugs are given. They are often given before surgery and continued afterwards in order to destroy any remaining cancer cells and prevent the sarcoma from spreading. Chemotherapy given in this way is called adjuvant chemotherapy.
If surgery is needed, it may be carried out at a specialist orthopaedic centre. Often, surgery can remove the tumour without causing too much damage. If the tumour is in one of the main bones of the arm or leg, however, it may be necessary to remove the whole limb (amputation) or part of the affected bone. If only part of the affected bone is removed, this is known as limb-sparing surgery.
Sometimes amputation of the limb is unavoidable if the cancer has affected the surrounding blood vessels and nerves. After amputation, a false limb will be fitted, and this will be regularly adjusted as your child grows. False limbs can work very well. It should be possible for your child to join in with normal activities and even sport.
There are several ways in which limb-sparing surgery may be done. It may involve:
replacing the bone with a prosthesis (a specially designed artificial part)
replacing the bone with bone taken from another part of the body (a bone graft).
After limb-sparing surgery, the child is usually able to use the limb almost normally. However, it's best not to take part in any contact sports, because any damage to the bone graft or prosthesis may require another major operation to repair or replace it. If the child is growing, the limb prosthesis will need to be lengthened from time to time as the bone grows. This may mean further short stays in hospital, although some prostheses can be lengthened during an outpatient procedure.
Radiotherapy treats cancer by using high energy rays that destroy cancer cells while doing as little harm as possible to normal cells. Ewing’s sarcoma responds very well to radiotherapy. It’s often used after chemotherapy and before or after surgery. If surgery is not possible - for example if the tumour.
Side effects of treatment for Ewing's sarcoma in children
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Treatment often causes side effects. Your child’s doctor will discuss this with you before the treatment starts. Any possible side effects will depend on the actual treatment being used and the part of the body that's being treated.
Side effects can include:
feeling sick (nausea) and being sick vomiting
increased risk of infection
bruising and bleeding
Radiotherapy can cause irritation or soreness of the skin in the area being treated and general tiredness. If your child is having surgery, the surgeon will explain the possible complications of the surgery that your child is having.
A small number of children may develop other side effects, sometimes many years later. These include a reduction in normal bone growth, infertility, a change in the way the heart and lungs work, and a small increase in the risk of developing a second cancer later in life. Your child’s doctor or nurse will explain all of this to you and will monitor your child carefully for any potential late side effects.
Clinical trials for Ewing's sarcoma in children
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Many children have their treatment as part of a clinical research trial. Trials aim to improve our understanding of the best way to treat an illness, usually by comparing the standard treatment with a new or modified version. Specialist doctors carry out trials for children's cancers. If appropriate, your child's medical team will talk to you about taking part in a clinical trial, and will answer any questions you have. Written information will be provided to help explain things.
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 a full understanding of the trial and what it means for your child to take part. Taking part in a research trial is completely voluntary and you'll be given plenty of time to decide if it's right for your child.
You may decide not to take part or to withdraw your child from a trial at any time. They will then receive the best standard treatment available.
Your child will have regular follow-up appointments, with x-rays or scans as necessary. Many children with Ewing’s sarcoma can be cured. Even if the tumour comes back, further treatment may be given successfully. Your child will be checked up on every few months for the first three years after treatment and then every six months for another two years.
If you have specific concerns about your child’s condition and treatment, it's best to discuss them with your child’s doctor who knows the situation in detail.
As a parent, the fact that your child has cancer is one of the worst situations you can be faced with. You may have many different emotions, such as fear, guilt, sadness, anger and uncertainty. These are all normal reactions and are part of the process that many parents go through at such a difficult time.
Your teams of doctors and nurses are there to help support your child and family as well as carry out treatments. It is important to discuss any difficulties you may be having with your treatment team.
We have more information about the emotional impact of caring for an unwell child and sources of help and support. There is also a page about the range of powerful emotions your child may experience throughout their illness, including how you can support your child.
Our booklet Katie’s Garden is a storybook for primary school-age children about a girl's experience of cancer.
CLIC Sargent offers practical support to children and young people with cancer or leukaemia, and to their families.
Children's Cancer and Leukaemia Group (CCLG)
CCLG coordinates research and care for children and their parents. There are 21 CCLG specialist centres for the treatment of childhood cancer and leukaemia, covering all areas of the UK and Ireland (there's a map of the centres on the website). The website also has information about CCLG, childhood cancer and leukaemia.
This information has been compiled using a number of reliable sources, including:
Voute PA, et al. Cancer in Children: Clinical Management. 5th edition. Oxford University Press. 2005.
Pinkerton, et al. Evidence-based paediatric oncology. 2nd edition.Blackwell Publishing. 2007.
Harman D, Gebhardt M. Treatment of the Ewing's family of tumours [online] Available from: http://www.uptodate.com/contents/treatment-of-the-ewing-sarcoma-family-of-tumors [Accessed November 2012]
Grimer et al. UK Guidelines for the Management of Bone Sarcoma. Sarcoma. 2010.
National Institute for Health and Clinical Excellence (NICE). Improving Outcomes for People with Sarcoma. [online] 2006. Available from: http://guidance.nice.org.uk/CSGSarcoma [accessed November 2012]
With thanks to: Dr Kate Wheeler, Consultant Paediatric 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.
We worked with the Children's Cancer and Leukaemia Group (CCLG) to write our information about children's cancer.