Ewing's sarcoma is a malignant (cancerous) bone tumor that affects children.
CausesEwing's sarcoma can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. It is uncommon in African-American, African, and Chinese children.
The tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. It may also develop in the skull or the flat bones of the trunk.
There are few symptoms. The most common is pain and occasionally swelling at the site of the tumor. Children may also break a bone at the site of the tumor after a seemingly minor injury (this is called a "pathologic fracture"). Fever may also be present.
The tumor often spreads (metastasis) to the lungs and other bones. Metastasis at the time of diagnosis is present in approximately one-third of children with Ewing's sarcoma. Rarely, Ewing's sarcoma can occur in adults.
SymptomsSymptoms include: -
- Pain at the site of the mass, with soft tissue swelling around the mass.
- In cases of metastatic disease (where the cancer has spread), patients may have general symptoms such as anorexia, fever, malaise, fatigue and weight loss.
- Other symptoms will be related to the specific location of the sarcoma.
- Fever (can occur)
- Pain at the site of the tumor
- Swelling at the site of the tumor (occasionally)
Exams and TestsThe exact causes of primary bone cancer are unknown. The development of Ewing's sarcoma may be related in some way to times of rapid bone growth, which may explain why more cases of Ewing's sarcoma are seen in teenagers. Like other cancers, it is not infectious and cannot be passed on to other people.
If a tumor is suspected, tests to locate the primary tumor and any spread (metastasis) often include: -
- Biopsy of the tumor
- Bone scan
- Chest x-ray
- CT scan of the chest
- Skeletal x-ray
How it is diagnosed ?
Usually you begin by seeing your family doctor (GP), who will examine you and may arrange tests or x rays. If a sarcoma is suspected, your GP will probably refer your child directly to a specialist hospital or bone tumour centre for further investigations. Many of the specific tests for diagnosing bone tumours, such as biopsies, need to be done by an experienced team using specialist techniques.
A variety of tests and investigations may be needed to diagnose a Ewing’s sarcoma. An x-ray of the painful part of the bone is usually able to identify a tumour, although sometimes this can be difficult to see. Other tests are taken to check whether the cancer has spread elsewhere. These include a chest x-ray, bone scan, bone marrow aspirate, and an MRI or CT scan.
TreatmentEwing’s sarcoma is treated by a combination of treatments which may include chemotherapy, surgery and radiotherapy. Treatment will depend on a number of factors, including the size and position of the tumour.
ChemotherapyChemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. This is an important treatment for most children with Ewing’s sarcoma, and has greatly improved the results of surgical treatment. It is often given before surgery. The course of chemotherapy will then continue after surgery, in order to destroy any remaining cancer cells and prevent the sarcoma from spreading outside the bone. Chemotherapy given in this way is called adjuvant chemotherapy.
SurgeryIf surgery is needed, it may be carried out at a specialist orthopaedic centre. Often, surgery can remove the tumour without causing too much damage. If the tumour is in one of the main bones of the arm or leg, however, it may be necessary to remove the whole limb (amputation) or part of the affected bone. If only part of the affected bone is removed, this is known as limb-sparing surgery.
AmputationAmputation of the limb is sometimes unavoidable if the cancer has affected the surrounding blood vessels and nerves. After amputation, a false limb will be fitted, and this will be regularly adjusted as the child grows. False limbs can work very well. It should be possible for the child to join in with normal activities and even sport.
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