People experience different symptoms depending on where their tumour arises. At the base of the skull, it can cause headaches, neck pain, and blurred or double vision. Occasionally it may affect facial sensation or movement, speech and throat functions. If it occurs in the vertebral bodies or the bones of the sacrum or coccyx (tail bone), it generally causes persistent back ache and may cause numbness or weakness of the arms and legs. Chordoma is slow-growing and, those tumours arising in the sacrum and lower back, in particular are often large by the time they are diagnosed. Furthermore, as the symptoms can mimic other common problems, such as back ache, and because chordoma is so rare, it is often takes a long time for a diagnosis to be made, especially as most GPs, general physicians and surgeons during their career will never meet a patient with chordoma.
A diagnosis of chordoma is generally suspected when a patient has undergone imaging (CT/CAT, MRI & PET Scanning ) following referral for their symptoms. A pathologist provides the definitive diagnosis when a biopsy (sample) of the tumour is taken.
The favoured treatment of chordoma is surgery to remove the tumour, although those situated at the base of skull may be treated with a form of targeted radiotherapy. Complete resection (removal of the whole tumour) during the first surgery provides the best chance for local control and long-term survival. Radiation therapy may also reduce the risk of recurrence after surgery and further prolong survival. However, as chordoma is always found close to important anatomical structures, such as the spinal cord, brainstem, nerves around the base of the brain and arteries they are difficult to treat and similarly it is difficult to remove the tumour completely without doing damage to these vital structures and requires highly specialised care. Furthermore, the tumour is composed of jelly-like material, making it easy for microscopic pieces of tumour to be left behind. For these reasons, recurrence of chordoma is very common. In up to 40% of patients, in the late stage of the disease, the tumour can spread to other parts of the body.
Patients with chordoma should be treated at specialist centres, whether they be private or through the NHS, where a team of knowledgeable physicians and health care workers are involved in a patient`s care. The team should involve radiologists to suggest the initial diagnosis (through CT/CAT, MRI & PET Scanning), pathologists to make the diagnosis on a small sample of tissue (biopsy) removed from the tumour, surgeons to operate, and oncologists to treat after surgery with radiotherapy and drug therapies as appropriate. A rehabilitation team also plays an important role in a patient’s recovery.
The hospital where a patient is treated will vary depending on the anatomic site of the chordoma. For instance, most chordoma in the sacrum and coccyx are likely to be treated at a national bone tumour referral centre of which there are five in the United Kingdom; Royal National Orthopaedic Centre in Stanmore Middlesex which is part of the London Sarcoma Service, the Royal Orthopaedic Hospital in Birmingham, the Nuffield Orthopaedic Centre Oxford, the Robert Jones and Angus Hunt Orthopaedic Hospital in Oswestry, and the Newcastle upon Tyne Hospitals NHS Foundation Trust.
Tumours in the rest of the spine will generally be treated by a team of spinal, orthopaedic and/or neurosurgeons in specialist units, with tumours at the base of the skull treated by neurosurgeons, again in specialist units such as the National Hospital for Neurology and Neurosurgery, Queen Square London.
Chordoma can occur in any age group, from infancy to the very elderly, although it is more commonly found in individuals over the age of 40. It occurs in approximately 1 in 800,000 people (incidence) and the number of people currently living with chordoma is approximately 8 per million of the population (prevalence). It is estimated that 500 people in the UK are currently living with the disease. It is slightly more common in men than women (1.6:1), and is even less common in the black African population. Chordomas at the base of the skull occur more commonly in younger people.
The vast majority of people who develop chordoma do not have a family history of the disease; that is, it occurs sporadically. The reason why a person develops chordoma is unknown.
There are a handful of families around the world in which a number of family members have developed chordoma: that is, they have a ‘familial form’ of the disease. This is extremely rare. If 2 members of a family have chordoma, it is recommended that other family members are investigated over a period of time to see if they are developing chordoma.
This information is provided for general guidance only and is not intended to be a substitute for specialist and professional medical advice, diagnosis or treatment. It is crucial to seek the advice of your GP, oncologist or other medical specialist with any questions you may have regarding your concerns and/or medical care.