Definition

Ewing’s sarcoma makes up 14% of all bone sarcoma diagnoses. It most commonly affects teenagers and young adults with the pelvis, thigh bone and shin bone being the most commonly affected areas.

In rare cases, Ewing’s sarcoma can develop in the soft tissue around the bone, this is called soft tissue sarcoma. It is sometimes called extraosseous sarcomaextra means outside, osseous means bone. Ewing’s sarcoma can also start in subcutaneous tissue, this means beneath the skin. 

Signs and symptoms

Symptoms of bone sarcoma can vary depending on the size and location of your tumour.

  • Bone pain, particularly occuring at night
  • A mass or swelling
  • Restricted movement in a joint

Symptoms can sometimes be confused with more common problems such as a sports injury or in children and young people, growing pains. 

Types of diagnostic scans

X-ray

Uses x-radiation to take images of dense tissuses inside the body such as bones or tumours.

CT

The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area.

PET

The Positron Emission Tomography (PET) scan shows up changes in tissues that use glucose as their main source of energy – for example, the brain or heart muscle. It involves an injection of a very small amount of a radioactive drug into the body. The drug travels to places where glucose is used for energy and shows up cancers because they use glucose in a different way from normal tissue. 

MRI

Magnetic Resonance Imaging (MRI) uses magnets to create an image of the tissues of the body.

Bone scan

Uses radioactive chemicals called radionuclides which are injected, swallowed or breathed into the body, to take images of bones. 

Histopathology

Examination of a tissue sample by a pathologist under a microscope to identify disease.

Blood test

Laboratory analysis of a blood sample.

Understanding the diagnosis

A diagnosis of bone sarcoma usually starts with a visit to your GP who will refer you for an x-ray. If a bone sarcoma is suspected following the results of the x-ray your GP will refer you to a specialist bone sarcoma centre. 

A specialist doctor will diagnose sarcoma through a series of tests. These may include:

  • Physical examination 
  • A scan – taking pictures of the inside of the body using ultrasound, x-ray, CT, EUS, PET or MRI
  • A biopsy – taking and testing a tissue sample

A diagnosis of bone sarcoma should be confirmed by a specialist sarcoma pathologist who will identify the type of sarcoma and the stage and grade of the tumour. Identifying the stage and grade of a cancer means your doctor can advise on the best course of treatment for you. It also describes the cancer in a common language which is useful when your doctor is discussing your case with other doctors or health professionals. The stage of a cancer is measured by how much it has grown or spread which can be seen on the results of your tests and scans. The results from a biopsy can tell the grade of the cancer. 

Grading

Low-grade means that the cancer cells are slow-growing and look quite similar to normal cells. They are less aggressive and less likely to spread. 

High-grade means the cancer cells are fast growing and look very abnormal. They are more aggressive and more likely to spread. 

Staging

Stage 1

The cancer is low-grade and has not grown outside the bone. Stage 1 is further divided into:

  • 1a The cancer is low-grade and is still completely within the bone it started in.
  • 1b The cancer is low-grade and has grown through the bone wall.

Stage 2

The cancer is high-grade and has spread beyond the bone. Stage 2 is further divided into:

  • 2a The cancer is high-grade and is still completely in the bone it started in.
  • 2b The cancer is high-grade and has grown outside the bone wall.

Stage 3

The cancer may be any grade and has spread to another part of the body, such as the lungs. 

Treatment

The National Institute for Health and Care Excellence (NICE) recommends that anyone with sarcoma should be referred to a specialist sarcoma team for diagnosis and treatment. There are five national bone sarcoma centres in England and Wales where all bone sarcoma patients should be treated. These are located in Newcastle, Manchester & Oswestry, Birmingham, Oxford and London.

People in Northern Ireland will usually travel to Birmingham for treatment. People in Scotland will be treated in Glasgow, Edinburgh, Aberdeen, Dundee and Inverness. This may mean you will have to travel some distance for your treatment. Some treatments like radiotherapy or chemotherapy may be given in your local hospital under the supervision of the specialist sarcoma centre.

Your case will be managed by a team of experts from a wide range of health care professions called a multidisciplinary team (MDT). Your MDT will include your key worker or sarcoma clinical nurse specialist, surgeon and other healthcare professionals involved in your care. 

Types of treatment

Chemotherapy

The first treatment method for Ewing’s sarcoma is chemotherapy. Chemotherapy uses anti-cancer drugs to destroy cancer cells. It is sometimes used before surgery to reduce the size of the tumour so it can be operated on and removed. This can be useful to treat large tumours and can prevent the need to have the limb either partially or fully amputated. Chemotherapy is also sometimes used after surgery. In this case, the aim is to kill off any local cancer cells which remain in the area of the tumour. The main types of chemotherapy used to treat Ewing’s sarcoma are doxorubicin, cyclophosphamide, ifosfamide, vincristine, dactinomycin and etoposide. These are often used in combination for example, VIDE which is a combination of vincristine, ifosfamide, doxorubicin and etoposide.

Surgery

Once the tumour has shrunk down with chemotherapy, the team treating you will consider whether or not any residual tumour should be removed surgically. In general, the best way to control the tumour is to operate. The surgeon will aim to take out the tumour and maintain as much limb function as possible. To achieve this margin of normal tissue, some patients will receive limb-sparing surgery. The aim of this surgery is to preserve the limb where the tumour is rather than amputating it. Limb-sparing surgery involves taking out the affected bone and replacing it with a bone graft (bone taken from another part of the body). Unfortunately, it’s not always possible to use limb-sparing surgery. Sometimes the cancer may spread from the bone to the nerves and blood vessels around it. If this happens the only way to treat cancer may be to remove part of the limb known as a partial amputation. Some people may need to have all of their limb removed. This is called a full amputation.

Tumours in the pelvis can sometimes be hard to remove with surgery. You may be given chemotherapy first, then surgery to get rid of all of the cancer. You may also receive radiotherapy after surgery to get rid of any cancer cells still in the area. Pelvic bones can sometimes be reconstructed after surgery, either by a bone graft or using a prosthesis. Unfortunately, in some cases pelvic bones and the leg they are attached to might need to be removed, this is called a hind-quarter amputation.

For a tumour in the lower jaw bone, the entire lower half of the jaw may be removed. This may be reconstructed using bones from other parts of the body. If the surgeon can’t remove all of the tumour, radiation therapy may be used as well.

If your tumour is in the spine or the skull, it may not be possible to remove all of the tumour safely. Sarcoma in these bones may require a combination of treatments such as chemotherapy, surgery, and radiation. 

Radiotherapy

Radiotherapy uses high-energy radiation beams to destroy cancer cells. It is effective in treating Ewing’s sarcoma and can be used before or after surgery. When used before surgery it aims to reduce the size of the tumour so it can be operated on and removed. When used after surgery, the aim is to kill off any local cancer cells that remain in the area of the tumour. It is sometimes used alongside chemotherapy, particularly if the tumour is in a site where removing it surgically is very difficult or surgery may be very disabling. 

After treatment

After treatment for sarcoma you may benefit from rehabilitation services. They can offer specialist advice and treatment that aids your recovery and helps you to deal with the effects of cancer and its treatment.

Rehabilitation services include:

Occupational therapy

Occupational therapists assess your ability to carry out daily living activities such as washing, dressing and meal preparation. They can also help you return to normal daily activities such as work, parenting, and leisure activities.

Physiotherapy

Physiotherapists help you return to as active a lifestyle as possible. They will help you strengthen your muscles and ensure your joints regain as much mobility as they can.

This may involve designing a special exercise programme, providing advice about managing tiredness or teaching you to use equipment to help you walk or to support your joints.

Dietary services

Dieticians assess whether you need any special diet and can advise on the most appropriate nutritional support to help you before, during and after treatment.

Orthotics and prosthetics

After surgery for bone sarcoma you may need aids to help you. For example, an orthotist can help by providing you with supports or splints. If you have had an amputation, a prosthetist can assess and fit an artificial limb. 

Rehabilitation usually starts after treatment. However, with bone sarcoma you may find that it helps to start rehabilitation earlier. Ask your sarcoma clinical nurse specialist or doctor to refer you to the rehabilitation team. You may find that you are not automatically offered a referral to rehabilitation services by your medical team, so it is important to ask at the earliest opportunity for a referral

Recurrence

A recurrence of cancer may appear in other parts of the body. This is called a metastasis or secondary cancer. In people with bone sarcoma, these secondary cancers may appear in the lungs. A chest x-ray is taken at follow-up appointments to look for secondary cancers in the lung.

Treatment for secondary cancers may involve surgery, radiotherapy or chemotherapy as appropriate; your treatment will be assessed on an individual basis. 

Emotional and practical support

Emotional support

TALKING ABOUT IT

There is no right or wrong way to feel or react to diagnosis; you may feel angry, sad or anxious about the future. You may also have concerns for how the news will affect your loved ones. You may find comfort in talking about your concerns with family members or friends.

TALKING TO OTHERS AFFECTED BY SARCOMA

If you do not feel comfortable discussing your concerns with family or friends, it may help to talk to other people who have been affected by sarcoma. There are a number of sarcoma support groups supported by Sarcoma UK around the country. These offer valuable support and information to patients, carers and family members, and provide the opportunity to meet with other people in the same situation.

Most groups are run by patients and carers working together with local sarcoma clinical nurse specialists or doctors. A typical meeting may include a talk from an invited speaker on a related topic, discussion and questions, informal chat with other group members, and refreshments.

There is also online support available for people affected by all types of sarcoma. Online support provides members with the opportunity to get in touch with other sarcoma patients or carers to discuss their concerns over a new diagnosis, treatment options or worries about the future.

Club HQ is a national support network for sarcoma patients who have had a hindquarter amputation. They offer information, mentoring, support, a private Facebook group and annual get-togethers to share and discuss experiences. New members are welcome to send their name, address, telephone and email to Club HQ, c/o Bone Cancer Research Trust, 10 Feast Field, Horsforth, Leeds LS18 4TJ. Tel: 0113 258 5934 Email: info@bcrt.org.uk 

TALKING TO A PROFESSIONAL

You may find it helpful to talk to your sarcoma clinical nurse specialist or doctor about your diagnosis; they will be able to answer any questions you may have about your condition. They may also be able to put you in touch with a counsellor for additional support, providing you with a safe, confidential place to talk about your concerns. Your GP will have access to local counselling services who can provide support to people with cancer.

Practical support

FREE PRESCRIPTIONS

Patients who are being treated for cancer can apply for a medical exemption (MedEx) certificate which will allow you to get free prescriptions. You can get an application form from your doctor and it will need to be countersigned by your GP or consultant. Prescriptions are free for all patients in Scotland, Wales and Northern Ireland.

BENEFIT ENTITLEMENT

Your sarcoma clinical nurse specialist should be able to advise you on the types of benefits you can claim or any special funding you can apply for. Your local Citizens Advice Bureau can also give you benefits information and many branches can help you fill out application forms. Macmillan Cancer Support have a number of benefit advisors who offer financial advice and support to cancer patients including advice about returning to work following cancer treatment.

Version 1

Date published: August 2015

Next review: August 2017