Chordoma is a rare type of bone cancer.
We have information on primary bone cancer which you may also find useful.
We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you’re having your treatment.
A chordoma is a rare type of cancer that develops from the notochord. The notochord forms the spine in a developing baby in the womb. After about six months, most of the notochord is replaced by the bones of the spine. However, small amounts of the notochord may remain and these can sometimes develop into a chordoma.
Chordomas can be found in any part of the spine. Around half occur at the bottom of the spine. About 2 in 5 (35-40%) occur at the base of the skull or in the bones running down the middle of the face. The rest affect the bones in the spine (the vertebrae).
Chordomas tend to be slow-growing and don’t often spread to other parts of the body. If they do spread, the most commonly affected places are the lungs, liver, nearby lymph nodes, bone and skin. As these tumours gradually grow, they can affect the surrounding areas of bone and soft tissue.
Chordomas can happen at any age, but mainly affect people aged 40-60. Fewer than 1 in 20 of all chordomas occur in people under the age of 20 and childhood chordomas are very rare. Chordomas, especially those that occur at the bottom of the spine, are more common in men than in women.
The exact cause of chordomas is unknown. Research is being carried out into possible causes.
Signs and symptoms of a chordoma
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It may take some time for a chordoma to be diagnosed, as symptoms often develop gradually. The symptoms a person has will depend on where the tumour is. If the chordoma starts in the spine, symptoms may include:
changes in bowel habit - for example, constipation
incontinence (poor bladder control)
changes in mobility - for example, weak legs
impotence (problems with erections).
If the chordoma starts in the base of the skull, symptoms may include:
changes in hearing
a feeling of dizziness (vertigo).
All of these symptoms are common to many other conditions and you will need further tests and investigations before an accurate diagnosis can be made.
How a chordoma is diagnosed
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Usually you begin by seeing your GP, who will examine you and may arrange any necessary tests or x-rays. For chordomas in the spine, your GP will probably refer you to a specialist surgeon called a spinal surgeon, who can carry out other investigations.
Your GP may also decide to refer you to a specialist hospital or bone tumour centre for further tests. This is because many of the tests for diagnosing bone tumours, such as biopsies, require experience and specialist techniques.
If you have a chordoma affecting the base of your skull, you may be referred to:
a neurologist (a doctor who specialises in treating illnesses of the brain and nervous system) or
a neurosurgeon (a doctor who specialises in operating on the brain).
Tests and investigations
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Your doctors need to find out as much as possible about the type, position and size of the tumour so they can plan your treatment. You may have a number of tests and investigations.
The doctor will examine you thoroughly. They will test your reflexes and the power and feeling in your arms and legs.
You will have a CT or MRI scan to find the exact position and size of the tumour.
CT (computerised tomography) scan
A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless and takes 10-30 minutes. CT scans use small amounts of radiation, which are very unlikely to harm you or anyone you come into contact with.
You may be given an injection of a dye, which allows particular areas of the inside of the body to be seen more clearly. For a few minutes, this may make you feel hot all over. If you are allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it is important to let your doctor know beforehand.
MRI (magnetic resonance imaging) scan
This test is similar to a CT scan, but uses magnetism instead of x-rays to build up a detailed picture of areas of your body. Before the scan, you may be asked to complete and sign a checklist. This is to make sure it's safe for you to have an MRI scan.
Before having the scan, you'll be asked to remove any metal belongings including jewellery. Some people are given an injection of dye into a vein in their arm. This is called a contrast medium and can help the images from the scan to show up more clearly.
During the test you will be asked to lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It’s painless, but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It's also noisy, but you'll be given earplugs or headphones.
To give an exact diagnosis, you may have a sample of cells taken from the tumour and examined under a microscope. This is called a biopsy. The biopsy involves an operation and you may have to stay in hospital for a few days. How it’s done will depend on the position of the tumour in the spine. Your doctor will discuss whether a biopsy is necessary in your case and exactly what the operation involves.
Treatment for a chordoma
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The treatment for a chordoma depends on a number of things, including your general health and the size and position of the tumour. The results of your tests will enable your doctor to decide on the best type of treatment for you. There are some risks associated with treatment and your doctor will discuss these with you.
As chordomas are very rare, they are usually treated at specialist hospitals by a team of doctors and other healthcare professionals. This means you may need to travel some distance for treatment.
Where possible, surgery is used to remove the tumour and nearby tissues that might contain cancer cells. The type of surgery you have, and your recovery, will depend on a number of factors. Your surgeon will discuss the different types of surgery with you in detail before any decision is made about your treatment. Before your operation, you can ask questions so that you know exactly what is involved. No operation or procedure will be done without your permission (consent).
In some situations, surgery to remove a chordoma of the spine may affect how your bowel and bladder work. Some people may need a colostomy or urostomy. If this is likely to be the case for you, your surgeon and specialist nurses will give you information about what to expect, and support to help you prepare for the surgery.
Sometimes it may be too difficult to remove a chordoma completely – for example, if the chordoma has spread into nearby areas of the body. In this situation, your doctor may recommend other treatments in addition to, or instead of, surgery.
Radiotherapy uses high-energy x-rays to destroy the cancer cells. It can be used:
after surgery to destroy any remaining tumour cells
alone to treat chordoma
with chemotherapy if surgery isn't possible
if the tumour comes back after initial treatment and further surgery is not possible.
Radiotherapy is also sometimes used to relieve symptoms, such as pain.
Newer methods of radiotherapy used for chordoma include stereotactic radiotherapy, image-guided radiotherapy (IGRT) and intensity-modulated radiotherapy (IMRT). These types of radiotherapy enable doctors to direct higher doses of radiation more accurately at the tumour and minimise the side effects of treatment.
Proton beam therapy is another type of radiotherapy that can be effective in treating chordoma. This treatment is currently unavailable in the UK. However, if it's suitable for you, the NHS may arrange for you to have it overseas. Proton beam therapy will hopefully be available in the UK from 2018. Your doctor can give you more information about this treatment.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy has not been shown to be very effective in treating chordomas. However, it may sometimes be used to try to control a chordoma that has come back (recurred) or has spread elsewhere in the body.
Cancer research trials are carried out to try to find new and better treatments for cancer. You may be asked to take part in a clinical trial. Your doctor will discuss the treatment with you so that you have a full understanding of the trial and what it means to take part. You may decide not to take part or to withdraw from a trial at any stage. If you do this, you will receive the best standard treatment available.
Targeted therapies use substances that target the differences between cancer cells and normal cells. Imatinib (Glivec ®) is a targeted therapy that is being tested to see if it is helpful in treating chordoma. There is also ongoing research looking into other targeted therapies for chordoma.
After your treatment is completed, you will have regular check-ups and possibly scans. These may continue for several years.
If you have any problems or notice any new symptoms between your check-ups, let your doctor know as soon as possible.
You may find the idea of a tumour affecting your spine extremely frightening. You may experience many emotions, including anxiety, anger and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their condition. Many people find it helpful to talk things over with their doctor or nurse, or with one of our cancer support specialists. Family members and close friends can also offer support.
Brain and Spine Foundation
The Brain and Spine Foundation offers an information service for patients, carers and healthcare professionals, an education programme for GPs and medical undergraduates, and workbooks for schools and colleges.
is a charity organised by UCL Cancer Institute Research Trust. It provides information for patients, family and friends and aims to raise money for research into the treatment of chordoma.
This section has been compiled using information from a number of reliable sources, including:
Bernstein M, Berger M. Neuro-oncology – The Essentials (2nd edition). 2008. Thieme
Snyderman C, et al. Chordoma and chondrosarcoma of the skull base. uptodate.com/contents/chordoma-and-chondrosarcoma-of-the-skull-base (accessed March 2014)
Stacchiotti S, et al. Phase II study of Imatinib in advanced chordoma. Journal of Clinical Oncology. 2012. Vol. 30: 9, 914-920.
Tobias J, Hochhauser D. Cancer and its management (6th edition). 2010. Wiley-Blackwell
Welch W, et al. Spinal cord tumors. uptodate.com/contents/spinal-cord-tumors (accessed March 2014)
Thank you to Professor Neil Burnet, Professor of Radiation Oncology who reviewed this information.
Thank you to all of the people affected by cancer who reviewed what you're reading and have helped our information to grow.
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