Ewing sarcoma usually occurs in the long bones, such as legs and arms, ribs, pelvis and spine. Very rarely, it can occur in the soft tissues. This is called extraosseous Ewing sarcoma.
The cells in a Ewing sarcoma have a specific genetic change within them that confirms the diagnosis. There are other Ewing sarcoma-like tumours that occur in bone or soft tissue, including primitive neuroectodermal tumour (PNET), BCOR or CIC-DUX rearranged tumours.
Causes
The exact causes of primary bone cancer are unknown. The development of Ewing 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.
It is important to remember that nothing you have done has caused your child’s cancer.
Signs and symptoms
Pain is the most common symptom of bone cancer. It is often worse at night. 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. This may cause a limp if in the leg or pelvis.
Bone cancer is sometimes discovered when a bone that has been weakened by cancer breaks after the child has a minor fall or accident. Occasionally, there may be fever or weight loss.
How Ewing sarcoma is diagnosed
A variety of tests and investigations are needed to diagnose Ewing sarcoma, including an x-ray and MRI scan of the painful part of the bone, a chest x-ray or CT scan and blood tests. A specialist doctor will remove a small piece of the tumour to look at under a microscope (biopsy). Other tests may be done, such as a bone scan, PET scan, or a bone marrow biopsy.
Any tests and investigations that your child needs will be explained to you.
Treatment
A combination of various treatments is used to treat Ewing sarcoma. These include chemotherapy, surgery and radiotherapy. Treatment will depend on a number of factors, including the size and position of the tumour.
Chemotherapy
Chemotherapy is the use of anti-cancer drugs to destroy cancer cells. This is a very important component of treatment for Ewing sarcoma and may make surgery more straightforward.
A combination of different chemotherapy drugs are given before surgery and continued afterwards in order to destroy any remaining cancer cells and prevent the sarcoma from spreading.
Radiotherapy
Radiotherapy treats cancer by using high energy rays that destroy cancer cells while doing as little harm as possible to normal cells. Ewing sarcoma responds very well to radiotherapy. It is often used after chemotherapy and before or after surgery. If the tumour is impossible to remove surgically, it is a good option.
Surgery
If surgery is needed, it will be carried out at a specialist orthopaedic bone tumour centre. The aim of surgery is to 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. Limb-sparing surgery will be performed if at all possible.
Amputation
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 work very well. It should be possible for your child to join in with normal activities and even sport.
Limb-sparing surgery
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 this type of surgery, children are often able to use their limb almost normally. However, it is best not to take part in any contact sports. This is because any damage to the bone graft or prosthesis may require another major operation to repair or replace it.
If your child is growing, the limb prosthesis will need to be lengthened from time to time as the bone grows. This may mean there are short stays in hospital, although some prostheses can be lengthened as an outpatient procedure.
Follow-up care
Once treatment has finished, the doctors will monitor your child closely with regular appointments to be sure that the cancer has not come back and there are no complications. After a while, you will not need to visit the clinic so often.
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.
Useful resources
This information is about Ewing sarcoma in children, aged 0-14 years.
Find out about bone cancer in teenagers and young adults (15 - 24 years)