Ewing’s sarcoma (paediatric)

Ewing’s sarcoma is named after Dr James Ewing, who first described the tumour in the 1920s. This type of cancer can develop anywhere in the body, but usually starts in a bone.

 The commonest bones involved are the pelvis, upper arm or thigh. Occasionally it starts in the soft tissue near a bone.

Who gets Ewing’s sarcoma?

About 30 children develop Ewing’s sarcoma in the UK each year. It is more common in teenagers and young adults, and boys compared to girls. The cause of Ewing’s sarcoma is unknown.

Signs and symptoms

The most common symptom is pain in the affected bone. There may be some associated swelling and tenderness. If the tumour develops in the soft tissue this normally presents as a lump. Occasionally the child may have a fever.

Tests / investigations

We will need to carry out some tests to find out as much as possible about the type, position and size of the tumour. These tests include:

  • X-ray of the affected bone to identify the tumour
  • chest X-ray to see whether the tumour has spread to the lungs
  • CT scan or MRI scan of the affected bone/tissue and chest to provide more information about the tumour and whether it has spread
  • biopsy – a small operation, usually carried out under general anaesthetic, to remove some tissue
  • bone marrow aspirate and trephine – these tests will tell us whether the tumour has spread to the bone marrow
  • PET/CT scan – this will tell us whether there is spread to other parts of the body

Staging

Staging refers to the size of the tumour and whether it has spread. Knowing the stage of the cancer helps the doctors decide on the most appropriate treatment for your child. Ewing’s sarcomas are divided into two groups. These are:

  • localised, where the tumour is found in only one part of the body
  • metastatic, where the tumour has spread to other parts of the body.

Treatment of Ewing’s sarcoma

Treatment for Ewing’s sarcoma involves chemotherapy, surgery and sometimes radiotherapy or a combination of all three. This depends on the type, stage and response to treatment.

Chemotherapy

A combination of anti-cancer drugs is used to destroy the cancer cells. The treatment is normally given every few weeks. The exact combination and number of courses depends on the stage and response of the tumour.

Some children will need high-dose chemotherapy with a stem cell transplant.

If your child takes part in a clinical trial, the treatment is explained in more detail in the specific trial information sheet.

An outline of the treatment will be provided in the form of a ‘road map’.

Surgery

An operation will be needed to remove the bone and tissue affected by the tumour. Because the tumours usually involve a bone it is often necessary to remove one or more bones. At the same time an artificial bone (prosthesis) can be put in place if necessary so that your child can lead a normal life. The prosthesis is made especially for your child, and may need to be altered as he/she grows.

Radiotherapy

Radiotherapy may be needed if surgery is not possible or when there is still some tumour left behind after the operation. See the Children’s Unit booklet Radiotherapy treatment for more details on this type of treatment.

Clinical trials

Clinical trials are medical research trials involving patients that are carried out to try and find new and better treatments. In cancer, clinical trials are most commonly used to try and improve different forms of treatment such as surgery, radiotherapy or chemotherapy.

The treatment being tested may be aimed at:

  • improving the number of people cured (for example, trying new types of surgery or chemotherapy)
  • improving survival
  • relieving symptoms of the cancer
  • relieving the side effects of treatment
  • improving the quality of life or sense of wellbeing for people with cancer.

Effects of treatment

About 60% of children with Ewing’s sarcoma are cured, however for the individual child this depends on the site of the tumour and whether it has spread.

General side effects of chemotherapy

Bone marrow suppression (myelosuppression)

Chemotherapy drugs decrease the production of blood cells by the bone marrow for a variable period of time. This results in low red blood cells (anaemia), low white blood cells (neutropenia) and platelets (thrombocytopenia). Your child may need blood or platelet transfusions and will be at increased risk of infections. The doctors and nurses caring for your child will tell you more about these side effects.

Nausea and vomiting

Some of the chemotherapy drugs used may make your child feel sick or vomit. We will give anti-sickness drugs at the same time to stop nausea and vomiting. These are usually very effective.

Hair loss

Temporary hair loss is common.

Specific drugs

For details of the side effects of individual drugs please see Macmillan specific drug information leaflets.

What are the possible long-term effects of treatment?

A small number of children develop side effects many years later because of the treatment they have received. These include problems with bone growth, impaired heart and lung function, impaired fertility and a small risk of developing a second cancer later in life. Teenage boys have the option to store sperm for use in later years.

About five years after treatment finishes we will transfer your child’s care to our long-term follow-up clinic. Your child will be seen at regular intervals in this clinic, indefinitely, so that we can help with any long-term effects of the treatment.

Clinical trials may also involve research aimed at understanding more about the tumour’s biology. You may be asked to allow us to do research on the tumour sample removed at surgery, or on blood samples.

Well-run clinical trials have led to a significant improvement in the treatment of children with cancer. If you are asked to consider entering your child into a clinical trial you will be given specific information about the trial before deciding whether to take part.