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 sarcoma – extra 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
Uses x-radiation to take images of dense tissuses inside the body such as bones or tumours.
The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area.
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.
Magnetic Resonance Imaging (MRI) uses magnets to create an image of the tissues of the body.
Uses radioactive chemicals called radionuclides which are injected, swallowed or breathed into the body, to take images of bones.
Examination of a tissue sample by a pathologist under a microscope to identify disease.
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.
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.
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.
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.
The cancer may be any grade and has spread to another part of the body, such as the lungs.
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
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.
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 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 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 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.
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.
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.
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.