What is Chordoma Spinal Cancer?

Chordoma is a very rare (1 in 800,000) type of primary bone cancer meaning it arises in bone (secondary bone cancer refers to those tumours that spread to bone from other parts of the body such as breast and prostate).

Chordoma arises in bones at the base of the skull (approximately 30%), the spine (approximately 20%) and the bone of the sacrum (lower back) and coccyx (the tail bone) (approximately 50%). Very rarely chordoma occurs at other sites of the body such as in other bones and in muscle, fat.

Most people who are diagnosed with a chordoma are in their fifties but children can also develop chordoma. There is a strong tendency for chordoma to affect people of white ethnicity (nearly 80% of cases) although it can affect other ethnicities too. In the vast majority of cases, chordoma arises sporadically, meaning it does not run in families. However, there are a handful of families in the world in which two or more family members have a chordoma meaning the disease is inherited.

Chordoma is thought to arise from embryonic tissue (notochordal cells), which normally disappears before birth, but can persist in about 20% of the population after birth. However, the vast majority of individuals with this persistent embryonic notochordal tissue, are completely unaware that they have it, and more importantly it is extremely rare for a chordoma to develop in those in whom it is present.

Chordoma is generally slow-growing and therefore people do not report the initially mild aches and pains associated with it to their doctor. It is for this reason that when patients go to see their doctor about their problems the tumour is often large by the time it is diagnosed. Because the symptoms can be so vague, such as lower back pain, which can be caused by more common conditions such as arthritis it can take some time before a chordoma is diagnosed. Chordoma at the base of the skull can cause headaches, or if it presses on the nerve controlling the eye muscles it may cause double vision, or other nerves of the head it may cause difficulties in swallowing. Chordoma at the base of the spine can grow quite large before causing symptoms or the symptoms can be non-specific such as back ache or difficulties passing stool.

Chordoma is one of a family of cancers called sarcoma, which include cancers of the bones, cartilage, muscles and other connective tissue. Sarcomas are rare representing only 2% of all cancers and so should be treated in specialist centres.

Frequently asked Chordoma questions

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.

More about chordoma symptoms

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.

More about chordoma diagnosis & treatment

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.

More about chordoma symptoms

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.

More about proton beam therapy

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.

More about chordoma diagnosis & treatment

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. Contact us to find out more.

Get in touch

“Hoping to keep well and carry on with life as long as possible and enjoy the grandkids.”

Read Mrs S' story

“I feel like I'm learning to walk all over again...”

Read Mr P' story

Want to make a donation?

You are the reason we can keep providing our vital supprt and funding. Individual donations and gifts may be made directly to Chordoma UK and enhanced through GIFT AID. Your fundraising can benefit from Chordoma UK`s charitable status enabling donations to be made through the Gift Aid scheme directly to the Chordoma UK bank account.

Donate now

Join our Mailing List

Please sign up to follow our ongoing story and events.


How your donation is spent

There is little funding available for research into this relentless bone cancer mainly because it is relatively rare, occurring in only 1 in 800,000 of the population. Without a focused approach to raising money for research into chordoma, little will change quickly.

Find out more

Get in touch

If you have any questions about Chordoma UK, or would like to talk to someone about cancer, send us an email and we'll get back to you as soon as we can.

Contact us


We all support each other

To share experiences, ask questions or vent your emotions and find others who understand.

Join us here