In this section
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.
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.
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 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%