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This information is about a rare type of primary bone cancer called a chordoma. It should ideally be read with our general information about primary bone cancer|.
A chordoma is a rare type of cancer that develops from the notochord. The notochord forms the early spine in a foetus as it develops inside 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, with around half occurring at the bottom of the spine. About 2 in 5 (35-40%) of chordomas 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.
Primary bone cancers are a rare type of cancer with fewer than 500 people diagnosed in the UK each year. Around 20 of these 500 people will have a chordoma.
Chordomas can happen at any age, but mainly affect people aged 40-60. Less 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 women.
Chordomas are believed to develop from pieces of notochord that, for some reason, did not break down as they should. Over many years, these harmless bits of notochord may transform and become cancerous, forming chordomas.
The exact cause of chordoma is unknown. Research| is being carried out into possible causes.
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:
If the chordoma starts in the base of the skull, symptoms may include:
All of these symptoms are common to many other conditions but it is generally possible for a bone specialist to diagnose chordomas.
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 an orthopaedic surgeon, who can carry out other investigations.
They 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).
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 and 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.
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, but takes 10-30 minutes. CT scans use small amounts of radiation, which will be very unlikely to harm you or anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.
You may be given a drink or injection of a dye that allows particular areas 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.
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 is 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, a sample of cells is sometimes taken from the tumour and examined under a microscope. The biopsy involves an operation and you may have to stay in hospital for a few days. The different ways of doing the biopsy 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.
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 consent (permission).
In some situations, surgery to remove a chordoma of the spine may affect how your bowel and bladder work. For some people, a colostomy or urostomy may be necessary. Your surgeon and specialist nurses will give you information about what to expect, and support to help you prepare, if this is likely to be the case for you.
Sometimes - for example if the chordoma has spread into nearby structures - it may be too difficult to remove the tumour completely. The doctor may recommend other treatments in addition to, or instead of, surgery.
Radiotherapy| treatment uses high-energy rays to destroy the cancer cells. It can be used after surgery to destroy any remaining tumour cells. It may be used alone to treat chordoma or with chemotherapy if surgery is not possible. It may also be given 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. This type of radiotherapy enables doctors to direct higher doses of radiation more accurately at the tumour and minimise the side effects of treatment.
Proton beam therapy is a type of radiotherapy that can be effective in treating chordoma. This treatment is currently unavailable in the UK, however, if it is suitable for you the NHS may arrange for you to have it overseas. 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, in some people it may help to control a chordoma that has recurred (come back) or has spread elsewhere in the body.
Biological therapies are man-made drugs that work by blocking (inhibiting) signals within cancer cells and preventing a series of chemical reactions that cause the cells to grow and divide. Imatinib (Glivec®)| is one such therapy that is being tested to see if it has any role in treating chordoma.
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.
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 these times, 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
Offers an information service for patients, carers and healthcare professionals, an education programme for GPs and medical undergraduates, and workbooks for schools and colleges.
Chordoma Support Group
This section is based on our Chordoma fact sheet and has been compiled using information from a number of reliable sources, including:
For answers, support or just a chat, call the Macmillan Support Line free (Monday to Friday, 9am-8pm)
If you have any questions about cancer, need support or just want someone to talk to, ask Macmillan.