Ewing sarcoma: symptoms, diagnosis and treatment

The NHS says the symptoms of primary bone cancer depend on the type and size of the cancer and where it is. Sarcoma UK says people often notice bone pain that does not go away and may be worse at night, a swelling or lump, and stiffness or problems moving a joint.

The NHS lists less common symptoms, and Macmillan notes that a primary bone cancer is sometimes found when a bone that has been weakened by the cancer breaks after a minor fall or accident, although the NHS calls breaking a bone a very rare symptom. Most of the time these symptoms are caused by other, more common conditions.

• Bone pain or tenderness, constant or coming and going, and often worse at night.

• A lump or swelling, which may not always be easy to see or feel.

• Problems moving around, a limp or difficulty moving a joint if the cancer is close to it.

• Feeling very tired even after a good night's sleep.

• A high temperature (fever) with sweating, and losing weight without trying (less common).

The NHS says to see a GP if you have pain or tenderness in a bone, particularly if it is worse at night, you notice a change that is not normal for you such as a lump, swelling or unexpected weight loss, or pain in your bones or joints is making it difficult to move around. You might not have bone cancer, but finding it early can mean it is easier to treat.

Macmillan says to tell your GP if the pain happens at night and when you are resting. The NHS says a GP may refer you to a specialist in hospital, which may be an urgent referral if you have certain symptoms, and that this does not definitely mean you have cancer.

Macmillan explains that sarcomas are rare cancers that develop in the supporting tissues of the body, such as bone, cartilage, tendons, fat and muscle, and that Ewing sarcoma is a type of primary bone cancer. Sometimes it starts in the soft tissue near the bone. These tumours are sometimes called primitive neuroectodermal tumours (PNETs) and are usually treated in the same way as Ewing sarcoma that starts in bone.

The CCLG says Ewing sarcoma is the second most common primary bone sarcoma in children and young people, with approximately 30 children in the UK developing it each year, and that the cells have a specific genetic change that confirms the diagnosis.

The NHS says it is not known what causes most primary bone cancers. Macmillan says research is happening to find out more about what may cause Ewing sarcoma. The CCLG says the development may be related in some way to times of rapid bone growth, which may explain why more cases are seen in teenagers.

The NHS lists factors that make bone cancer more likely in general: having had radiotherapy or chemotherapy for cancer in the past, particularly in childhood, an inherited condition such as hereditary retinoblastoma, Li-Fraumeni syndrome or Werner syndrome, or another bone condition such as Paget's disease of bone or a non-cancerous bone tumour.

Macmillan says you usually start by visiting your GP, who will check the area and may arrange tests or X-rays, and will refer you to a specialist sarcoma centre for tests if they are not sure what the problem is or think you may have a bone cancer. The NHS says it should take about 2 weeks for bone biopsy results to come back, but sometimes longer.

Bone biopsies and other tests to diagnose bone cancer are done by sarcoma specialists. Macmillan says tests on the tissue sample may look for genetic changes in the sarcoma cells, which helps tell the type of sarcoma and which treatments are suitable.

Macmillan says that because Ewing sarcoma is rare it is treated by a team of doctors and other professionals at a hospital with a specialist sarcoma treatment centre. A multidisciplinary team (MDT) discusses the best treatment, which depends on the position and size of the cancer, whether it has spread, its grade and your general health. Most people have a combination of treatments.

The CCLG says Ewing sarcoma usually occurs in the teenage years and is more common in boys, in long bones such as the legs and arms, the ribs, pelvis and spine. Pain is the most common symptom and is often worse at night, and swelling may appear if the tumour is close to the surface, causing a limp if in the leg or pelvis. Occasionally there may be fever or weight loss.

Treatment for children combines chemotherapy, surgery and radiotherapy, with surgery carried out at a specialist orthopaedic bone tumour centre. The CCLG says limb-sparing surgery is carried out if at all possible, and that if amputation is unavoidable a false limb is fitted and adjusted as the child grows, and children should be able to join in normal activities and even sport. After limb-sparing surgery it is best not to take part in contact sports, because damage to a bone graft or prosthesis may need another major operation.

Macmillan says that after you finish treatment you will have regular check-ups for a few years, including chest X-rays, and may have scans and blood tests. Tell your cancer doctor as soon as possible about any new symptoms between appointments.

A small number of people have side effects that continue after treatment, which vary with the size and position of the tumour and the treatment, and some may develop many years later. Macmillan says rehabilitation can help and treatment may affect fertility, so it is important to talk to your doctor about fertility before treatment starts. Sarcoma UK and the Bone Cancer Research Trust provide information and support.

On the NHS, a GP refers suspected bone cancer to a bone specialist or sarcoma centre. Diagnosis and treatment are carried out by sarcoma multidisciplinary teams, and a clinical nurse specialist is your main point of contact. The NHS says Sarcoma UK has a free support line.

Because Ewing sarcoma is so rare, care is concentrated in specialist centres. If you want a second opinion or private assessment, it is sensible to choose a centre with a sarcoma MDT, and to share the results with your NHS team so that treatment planning is not delayed.