This tumour is rare with an incidence of approximately one in two million. It is mainly seen in the long bones of the arms, legs, ribs, pelvis and vertebrae
The tumour tends to occur in young people and is rarely seen befor the age of 5 years or after the age of 30 years, with the commonest age of onset between 10 and 20 years. In some cases, mild trauma has occurred to the area where the tumour originates from.
There is no association with family inheritance although there are rare reports of the tumour occurring in siblings. Genetics do play a role as the tumour is more common in the white population than Afro-Caribbean or Chinese populations.
There are no obvious associations with diet.
Symptoms and signs
Pain and swelling are the common presenting features which may be accompanied by redness of the overlying skin. In some cases there is a mild fever as well which may initially suggest an underlying bone infection rather than a tumour.
Surgery is the ideal option but this may not be possible due to spread of the tumour. In addition to surgery, radiotherapy is sometimes used to try and eradicate any remaining tumour cells. When the tumour has spread, chemotherapy is given to try and control the tumour.