Ewing sarcoma

Ewing sarcoma is a type of primary bone cancer, which is also called bone sarcoma. Sarcomas are rare cancers that start in the supporting tissues of the body. These tissues include:

• bone
• cartilage
• tendons
• fat
• muscle

There are 2 main types of sarcoma:

• bone sarcomas (also called primary bone cancer)
• soft tissue sarcoma

Primary bone cancer can start in any bone in the body. It can also start in the soft tissue near bones.

The bones most often affected by Ewing sarcoma are the:

• pelvis
• thigh (femur)
• shin (tibia)
• ribs
• shoulder blade

Ewing sarcoma is most often found in teenagers and young adults, but it can happen at other ages. It's slightly more common in men than in women.

Sometimes the cancer starts in the soft tissue near the bone instead. This is called extraosseous Ewing sarcoma. "Extra" means outside and "osseous" means bone. You may also hear it called soft tissue Ewing sarcoma, and sometimes these cancers are called primitive neuroectodermal tumours (PNETs).

These cancers are often treated in the same way as Ewing sarcoma that starts in the bone.

Pain is the most common symptom of bone cancer, and that includes Ewing sarcoma. But the symptoms can differ depending on which part of the body the cancer is in.

The area may be:

• swollen
• painful when touched

Primary bone cancer is sometimes found when a bone breaks after a minor fall or accident. The cancer has weakened the bone, which is why it breaks so easily.

Most of the time, these symptoms are caused by other conditions that are far more common than bone cancer. Still, if you have any swelling or unexplained bone pain, get it checked by your doctor.

Tell your doctor if the pain comes at night and when you're resting.

The exact causes of primary bone cancer aren't known. Research is going on to find out more about what may cause Ewing sarcoma.

Macmillan Cancer Support has information on the causes of bone cancer.

If you have symptoms of Ewing sarcoma, you'll usually start by seeing your doctor. They'll check the affected area and your general health, and they may arrange some tests or X-rays.

Your doctor will refer you to a specialist sarcoma centre for tests if they:

• aren't sure what the problem is
• think you may have a bone cancer

Your doctor may ask for an X-ray to look at an area of bone for cancer, including Ewing sarcoma. The X-ray gives a picture of the bone. From it, the doctor can get an idea of where the cancer may have started, or what type of bone cancer it is.

An MRI scan uses magnetism to build up a detailed picture of areas inside your body.

In a bone biopsy, the doctor takes a small sample of tissue from the bone. The sample is then looked at under a microscope to check for cancer.

The bone marrow is the spongy material inside your bones, where blood cells are made.

If Ewing sarcoma is suspected or has been diagnosed, the doctor will take a small sample of bone marrow. This is because Ewing sarcoma can sometimes spread to the bone marrow.

The sample is usually taken from 1 side of the hip bone (pelvis). Sometimes the doctor takes a sample from the other side as well.

A CT scan takes a series of X-rays and puts them together to build a three-dimensional (3D) picture of the inside of your body. If you have Ewing sarcoma, you may have a CT scan of the affected bone and also of your chest.

A PET-CT scan, which doctors may use when looking into Ewing sarcoma, combines two scans in one. The first is a CT scan, which takes a series of X-rays to build up a three-dimensional (3D) picture. The second is a positron emission tomography (PET) scan.

Bone biopsies and the other tests used to diagnose bone cancer, including Ewing sarcoma, are done by sarcoma specialists.

The tissue sample may be tested for genetic changes in the sarcoma cells. This is sometimes called cytogenetic testing.

The sample is also tested for any proteins the cancer cell might be producing. This can help your doctor tell what type of sarcoma you have and whether certain treatments are suitable for you.

Waiting for test results can be a difficult time, and more information and support can help.

Ewing sarcoma is rare, so it's treated by a team of doctors and other health professionals at a hospital with a specialist sarcoma treatment centre.

Your test results will be discussed by a team of specialists. If the tests show bone cancer, a team of specialist doctors and other professionals will meet. This is called a multidisciplinary team (MDT), and they'll discuss the best possible treatment for you.

After the MDT meeting, your cancer doctor or specialist nurse will explain the treatment options and the possible side effects. They'll also talk with you about the things to consider when you make a decision.

The treatment you have depends on a number of things, including:

• the position of the cancer
• the size of the cancer
• if it has spread (its stage)
• the grade of the cancer
• your general health

The main treatments for Ewing sarcoma are surgery, radiotherapy and chemotherapy. You usually have a combination of these treatments.

You may be offered some treatments as part of a clinical trial.

If it can be done, doctors use surgery to remove Ewing sarcoma. The aim of the operation is to take out the affected area of bone. Some people may have reconstructive surgery at the same time.

If the bone sarcoma is in an arm or leg, it's usually possible to remove it with limb-sparing surgery.

Very rarely, the best treatment is to remove the whole of the affected limb (amputation).

Chemotherapy for Ewing sarcoma uses anti-cancer (cytotoxic) drugs to destroy cancer cells. It's often given before surgery to shrink the tumour and support better results from the operation.

You'll usually have more chemotherapy after surgery to destroy any remaining cancer cells.

Sometimes it's used to shrink large cancers, so that a limb doesn't have to be removed (amputation).

Radiotherapy uses high-energy rays to destroy cancer cells, while harming normal cells as little as possible. Ewing sarcoma responds very well to it. Doctors may use radiotherapy together with surgery, either before or after the operation.

When surgery isn't possible, radiotherapy may be given along with chemotherapy. This is called chemoradiation.

Radiotherapy can also be used to treat Ewing sarcoma that has spread to the lung.

Sometimes a type of radiotherapy called proton beam therapy (PBT) is used.

Once your treatment for Ewing sarcoma is over, you'll have regular check-ups for a few years. These will include chest X-rays, and you may have scans and blood tests too.

If you have any problems, or notice any new symptoms between your follow-up appointments, tell your cancer doctor as soon as possible.

A small number of people with Ewing sarcoma still have side effects after treatment ends. How much this happens depends on the size and position of the tumour, and on the treatment you had. You may have rehabilitation to help you recover and manage any side effects.

Sometimes side effects only show up after treatment has finished, and some may appear many years later. Your cancer doctor or specialist nurse will explain more about any possible late effects and what can help to manage them. If side effects aren't getting better, always tell them.

Ewing sarcoma, like other cancers, and its treatment can sometimes affect your sex life. There are ways to look after your sexual wellbeing and to manage any problems that come up.

Your body may change with cancer and its treatment, and that can affect how you see yourself. There are things that can help you cope with these changes.

Treatment for sarcoma may affect your fertility. If you're worried about this, talk with your doctor before you start treatment.

Macmillan Cancer Support, a UK charity, has more information about:

• getting pregnant after treatment
• making someone else pregnant after treatment
• LGBTQ+ people and cancer treatment

Even if you already live a healthy life, you may want to make a few positive changes once your treatment for Ewing sarcoma is over. Small things, like eating well and staying active, can improve your health and wellbeing and help your body recover.