Ewing sarcoma is a rare type of cancer that affects bones or the tissue around bones.

It mainly affects children and young people, with most cases diagnosed in people aged 10 to 20. It's more common in males than females.

Symptoms of Ewing sarcoma

The main areas affected by Ewing sarcoma are the:

• legs (most often around the knee)
• pelvis
• arms
• ribs
• spine

Symptoms include:

• bone pain – this may get worse over time and may be worse at night
• a tender lump or swelling
• a high temperature (fever) that doesn't go away
• feeling tired all the time
• weight loss

Affected bones may also be weaker and more likely to break. Some people are diagnosed after they have a fracture.

Tests for Ewing sarcoma

Ewing sarcoma can be difficult to diagnose because it's quite rare and the symptoms can be similar to lots of other conditions.

Several tests may be needed to diagnose the cancer and see where it is in the body.

These tests may include:

• an X-ray
• blood tests
• an MRI (magnetic resonance imaging) scan, a CT (computerised tomography) scan or a PET (positron emission tomography) scan
• a bone scan – after having an injection of a slightly radioactive substance that makes the bones show up clearly
• a bone biopsy, where a small sample of bone is removed using a needle or during a small operation so it can be checked for signs of cancer and certain genetic changes associated with Ewing sarcoma

Treatments for Ewing sarcoma

Treatment for Ewing sarcoma often involves a combination of:

• surgery to remove the cancer
• chemotherapy – where medicine is used to kill cancer cells
• radiotherapy – where radiation is used to kill cancer cells

Most people have chemotherapy to shrink the cancer, followed by surgery to remove as much of it as possible and then further chemotherapy to kill any leftover cancer cells.

Radiotherapy is often used before and after surgery, or it may be used instead of surgery if the cancer can't be removed safely.

Your care team will recommend a treatment plan they think is best. Talk to them about why they have suggested it and ask about the benefits and possible risks involved.

As Ewing sarcoma is rare and requires complicated treatment, you should be treated by a team specialising in the condition. If it affects your bones, surgery should be carried out at a specialist bone cancer centre.

Surgery

There are three main types of surgery for Ewing sarcoma:

• removing the affected bone or tissue – this is called a resection
• removing the bit of bone containing cancer and replacing it with a piece of metal or a piece of bone taken from another part of the body – this is called a limb-sparing surgery
• removing all or part of an arm or leg – this is called an amputation 

The best option depends on where the cancer is in the body and how big it is.

Ask your care team which type of surgery they recommend and what care might be needed afterwards – for example, if you'll need a prosthetic limb and support to help you regain the use of the affected limb.

Outlook for Ewing sarcoma

Ewing sarcoma can spread to other parts of the body quite quickly. The earlier it's diagnosed, the better the chance there is of the treatment being successful.

It can be cured in some cases, but this might not be possible if the cancer has spread.

The cancer can also come back after treatment, so regular check-ups will be offered to look for any signs of this.

Overall, more than half of people with Ewing sarcoma live at least five years after being diagnosed, but this can vary quite a lot.

Speak to your care team if you would like to know the chances of treatment being successful for you or your child.

More information and support

Being told you or your child has cancer can be very upsetting and overwhelming.

As well as the support from your care team, you may find it useful to get information and advice from charities and support groups.

Two of the main organisations for people with Ewing sarcoma are:

• Sarcoma UK – which provides information on Ewing's sarcoma as well as general support for people with sarcoma
• Bone Cancer Research Trust – which has detailed information on Ewing sarcoma and provides support for people with bone cancer

Other good sources of information and support include:

• Macmillan Cancer Support
• Cancer Research UK
• CLIC Sargent
• Children's Cancer and Leukaemia Group

There may be a clinical trial suitable for your or your child. You can ask your doctor for further information and can find out about trials for Ewing sarcoma at the Euro Ewing Consortium and the Cancer Research UK clinical trials page.