What is Ewing Sarcoma?

Ewing sarcoma accounts for approx 10-15% of all primary malignant bone tumours and occurs most often in the bones of the trunk (pelvis, scapula, and ribs) or in the mid-shaft of long bones such as the femur.

Ewing sarcoma rarely occurs in children under 5 years, the peak incidence is between 10 and 15 years.
It was originally thought that Ewing sarcoma only occurred in the bone, however other tumours were found within the soft tissues and is thought to be similar microscopically. These include extraosseous Ewing sarcoma and peripheral primitive neuroectodermal tumour (PNET) and are now grouped together as the Ewing sarcoma family of tumours.

Signs and Symptoms

As with other primary malignant bone tumours, the most common symptoms are increasing, persistent pain and swelling of the affected area with impairment of function.

The patient usually presents with a palpable, painful, and tender swelling, rapidly
increasing but quite variable in size.

Depending on the location of the tumour a variety of symptoms may be reported and occasionally a fever develops.

Diagnosis

If a child has pain in a bone an x-ray will probably be taken. If this suggests there is a tumour, a biopsy will be carried out to determine exactly what sort of tumour it is. Further x-rays and an MRI scan of the limb may give an idea of the extent of the tumour and may be done prior to the biopsy. Blood tests will be done and a chest x-ray, bone scan and bone marrow aspiration may also be carried out to investigate whether the cancer has spread to other parts of the body.

Treatment

Treatment for Ewing sarcoma usually involves all three modalities that being chemotherapy, surgery and or radiotherapy. Intensive chemotherapy is used initially to reduce the size of the tumour and enable subsequent surgery. Surgery is carried out if the tumour is in a bone, which can be removed, without causing cosmetic or functional damage, limb preservation or bone grafts are often possible. Radiotherapy may be used when tumours are not fully resectable, Since the introduction of using these 3 modalities for Ewing sarcoma the long-term survival rate has improved. Prognosis is dependant on the extent of the disease at initial presentation, size of the tumour and response to therapy, however for localised disease the disease free survival rate would be approx 50-70%