A rare primary bone tumour that affects the base of the skull, vertebral bones (bones of the spinal column) or lower back (sacrum, and coccyx - tail bone).

A chordoma that arises in a bone called the clivus, which forms part of the base of the skull.

Chordoma arising in the sacrum or coccyx (tail bone) at the base of the spine.

Symptoms vary depending on the location of the cancer within the spine. Chordoma develops in the bones of the spine (spinal vertebrae), so often causes non-specific symptoms such as back pain. As chordoma grows it can affect the nerves and cause sensory symptoms (tingling, or loss of sensation) or weakness.

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The reason why a person develops chordoma is unknown. In a small number of individuals with chordoma it runs in families but this is very rare.

Appearances on MRI vary between tumours and the appearances are best recognised and reported by radiologists who specialise in bone tumours. Chordoma tends to look “brighter” on the T2 images, where fluid is brighter as well, and “darker” on T1 images where fluid is darker as well. Chordoma tends to take up the contrast dye that is sometimes given but this can be variable. Different hospitals may also perform other types of specialist MRI images to see the tumour more clearly and make a diagnosis.

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MRI showing scan of a tumour.

A tumour means that cells which are growing in an uncontrolled manner and they form a lump). ‘Spinal tumour’ is a very loosely used term. It can refer to a tumour in the bones of the spine, but also tumours in the spinal cord (ependymomas) and in other structures such as the covering of the spinal nerves (meningiomas). Spinal tumours can be benign, such as a Benign Notochordal Cell Tumour, or they can be cancerous such as chordoma.

Tumours that spread to the spinal bones from another site of the body are referred to as ‘spinal metastases’.

Primary spinal cancer, is cancer that arises in or around the spinal cord or in the bones of the spine, is rare and includes different tumours such as meningioma, ependymoma and chordoma. Chordoma arises in 1 in 800,000 people per year.

Secondary spinal cancers are more common that primary spinal cancers and these spread from many cancers including those that arise in the lung, breast and prostate.

People experience different symptoms depending on where their tumour arises. The most common signs however are very non-specific and can include ‘ache and pains’ of different severity. Many people have back pain which often resolves with time whereas the pain with chordoma does not resolve and often gets worse. People can also exhibit neurological changes, this reflects that nerves are being compressed by the tumour but similar symptoms can be caused by other very common conditions such as arthritis and osteoporosis meaning that chordoma may take a long time to diagnose.

Chordoma at the base of the skull, 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 (lower back) or coccyx (tail bone), it generally causes persistent back ache and may cause numbness or weakness of the arms and legs and impact on bowel or bladder function.

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 given chordoma is so rare, it is often takes a long time for a definitive diagnosis to be made, as most GPs, general physicians and surgeons during their career will never meet a patient with chordoma.

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Proton beam therapy is a special type of radiotherapy that uses high-energy protons rather than high-energy (photon) radiation to deliver a dose of radiotherapy. Proton beam therapy can be more effective than regular radiotherapy as it can be delivered precisely to where it is needed. This reduces the risk of damage to the surrounding tissue or vital organs and delivers a high dose where it is required.

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Patients with chordoma should be treated at specialist centres, whether they be treated in the private sector or in 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.

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