Chordomas are rare tumours with fewer than 200 new cases being diagnosed each year in the UK.
They develop from remnants of the notochord (hence the name 'chordoma'). The notochord is a fibrous band which develops in the human embryo as a fore-runner of the spine. This means that chordomas occur close to, or within, the spinal column.
About half of all chordomas occur close to the top of the spine, where it joins the base of the skull. Other sites are the bones of the neck and the lower part of the back, the middle of the spine is very seldom affected.
Chordomas are very slow-growing tumours but without treatment they will continue to grow relentlessly, steadily damaging and destroying the bone and nerves around them. About 1 in 10 chordomas will spread through the blood stream to form secondary tumours in other parts of the body.
Because they are slow-growing and rare it is often some time before they are diagnosed. Many people with chordomas will have symptoms for more than a year before the cause is discovered.
The commonest symptom is pain due to the damage caused to the surrounding tissues.
Whenever possible surgery is the best treatment for a chordoma. Even if the tumour cannot be removed completely than taking away as much of it as possible is still important in helping to control the symptoms and slow the progress of the disease.
Often radiotherapy is given after surgery and this does seem to help. Sometimes if the chordoma is in a site where surgery would be too dangerous to consider, radiotherapy can be used as an alternative.
Occasionally there may be a case for giving a very special type of radiotherapy called proton beam treatment. This is not available in the UK but can be done at one hospital in France and one in Boston. If specialists feel this type of treatment is necessary, then it can be funded by the NHS.
Surgery and radiotherapy can cure or control most chordomas for many years. But the tumours do have a tendency to come back and continue to grow slowly, so regular check ups will always be needed.