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Malignant Bone Tumours
Although malignant tumours involving bone are fairly uncommon, representing about 5% of cancers in childhood and adolescence, they are of special significance. They occur most frequently in the adolescent age group where independence, self esteem, body image, active life and healthy competition with peers are important. Treatment for the cancer may involve amputation with its obvious dramatic impact at the physical and psychological level. The effects of local therapy may also be significant. There are two major types of bone cancer, Osteogenic Sarcoma and Ewing's Tumour (or Sarcoma).
Osteogenic Sarcoma:
Osteogenic sarcoma is about three times more common than Ewing's tumour, and occurs
usually between the ages of 10 and 25, and develops especially in long bones, most
commonly above or below the knee. The next commonest site is the upper arm (Humerus). The
tumour develops usually towards the growing end of a long bone and may become evident
because of swelling, pain, restricted movement or a limp, and occasionally with a painfree
fracture. The diagnosis is suspected strongly on the basis of clinical findings and the
use of x-rays, bone scans, and other methods of imaging, which also assist in defining the
extent of the disease. A biopsy of the tissue is required to make a diagnosis.
Unless treated, the tumour may be expected to enlarge and spread, usually to the lungs, within 12 months. Therefore, a programme of treatment is important, and the current plan is to give chemotherapy with multiple drugs (such as Methotrexate, Adriamycin and Cisplatinum) preoperatively for about 2 months. This treatment allows time for shrinkage of the tumour. Operation is then undertaken; it may comprise amputation above or below the knee (depending on the site of the tumour) or sometimes removal of tumours with insertion of a prosthesis to replace the missing bone (endoprosthesis). Insertion of an endoprosthesis is often attempted in tumours of the Humerus. Chemotherapy is continued for at least several months after operation, and the likely overall cure rate is 70%. Spread of the disease (metastases), usually to the lungs, may be present at diagnosis or occur subsequently, when other treatment may include additional chemotherapy and operative removal of metastases.
Ewing's Sarcoma:
Ewing's sarcoma is less common, occurs maximally in the 5-15 years age group, and is sited
mainly towards the middle or shaft of the bones. While most common in long bones, about
40% occur in flat or less accessible bones such as the pelvis, or vertebrae (backbone).
Sometimes especially in the pelvis, the tumour may grow to quite a large size before being
detected. The symptoms, clinical findings and investigations are similar to those of
Osteogenic Sarcoma. Chemotherapy usually comprises the use of several drug combinations
which may include: Adriamycin, Cyclophosphamide or Ifosphamide, Vincristine, Etoposide and
sometimes Platinum, and radiotherapy is usually given to the primary tumour. Sometimes,
surgical removal of the primary tumour is undertaken. Response to treatment is usually
good, but recurrence or metastases may develop, requiring further treatment. Over 60% of
patients are likely to be cured of the tumour.
General Management For Patients With Bone
Tumours:
Apart from treatment with drugs, operation or radiotherapy; physical, medical and
psychosocial support is very important. Amputees require re-education in walking habits
and assistance with artificial limbs. Adjustment to the altered lifestyle may seem
extremely difficult. Despite this, the number of patients who achieve well beyond
expectation both in the sporting and academic arenas is most impressive. Apart from
regular check ups including x-rays and scans in the first two to three years, patients
need to be followed up over long periods to ensure that they do not develop any undue late
effects from therapy which could include risks of infertility, second tumours or local
cosmetic effects. At long term follow up clinics, advice and reassurance concerning the
patient's worries can usually be given.
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