This information is about a rare type of primary bone cancer called a chordoma. You may also find it helpful to read our general information about primary bone cancer.

Primary bone cancer is a rare type of cancer that starts in the bone. Fewer than 500 people are diagnosed with it in the UK each year. About 20 of these 500 people will have a type of bone cancer known as a chordoma.

A chordoma is a tumour 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 can happen at any age, but mainly affect people between 40 and 60. Less than 1 in 20 of all chordomas occur in people under the age of 20 and childhood chordomas are very rare. Chordomas are more common in men than women, especially those that occur at the bottom of the spine.

Chordomas tend to be slow-growing, and don’t often spread to other parts of the body. If they do spread, the most common places chordomas can spread to are the lungs, nearby lymph nodes, liver, bone and skin. As these tumours gradually grow, they can affect the surrounding areas of bone and soft tissue. This can cause some of the symptoms listed in the section 'Signs and symptoms' below.

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 and what increases the risk of developing it is unknown. We do know they are not caused by any injury to the bone, or by an inherited faulty gene.

It may take some time for a chordoma to be diagnosed, as symptoms often develop gradually. If the chordoma starts in the spine, symptoms may include:

• pain
• changes in bowel habit
• incontinence
• impotence
• numbness
• changes in mobility (movement)

If the chordoma starts in the base of the skull, symptoms may include:

• headache
• double vision
• facial pain
• changes in hearing
• difficulty swallowing.

The symptoms a person has will depend very much on where the tumour is.

Tumours in the lower spine can cause pain, constipation, and bleeding from the rectum. If the tumour presses on nerves this may result in numbness, difficulty passing urine, and incontinence. Men may develop impotence. Other problems include weakness and difficulty coordinating movement. Tumours higher in the spine may cause pain and difficulty swallowing.

Chordomas at the base of the skull can cause double vision, headaches, difficulty swallowing and vertigo. Numbness, facial pain and changes in hearing can also occur.

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 illness of the brain) and a neurosurgeon (a doctor who specialises in operating on the brain).

The doctor at the hospital will take your full medical history before doing a physical examination. This will include an examination of the painful bone to check for any swelling or tenderness. You will also probably have a blood test to check your general health.

To plan your treatment, the doctors need to find out as much as possible about the type, position and size of the tumour. They do this by carrying out a number of tests and investigations.

CT (computerised tomography) scan This is a type of x-ray, which builds up a three-dimensional picture of your bones and soft tissue. The scan is painless, but you will have to lie still for about 10–30 minutes. It may be used to find the exact position and size of the tumour.

Before having the scan, you may be given an injection of a special liquid to make the picture clearer. This may make you feel hot all over for a few minutes. If you have asthma, or are allergic to anything (especially iodine) you should tell your doctor - and the person doing the test - before you have the injection.

MRI (magnetic resonance imaging) scan This test is similar to a CT scan, but uses a strong magnetic field instead of x-rays to build up cross-sectional pictures of your body.

During the test you will be asked to lie very still on a couch inside a long tube for up to an hour. It is painless, but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It is also noisy, but you will be given earplugs or headphones to wear.

Before the test, some people are given an injection of dye into a vein in their arm to improve the image, but this does not usually cause any discomfort.

The cylinder is a very powerful magnet, so before entering the room you should remove any metal belongings. People who have heart monitors, pacemakers, or some types of surgical clips cannot have an MRI scan because of the strong magnetic field.

The type of treatment you have will depend on a number of things, including the position and size of the cancer, whether it has spread, and your general health.

As chordomas are very rare they are usually treated at specialist hospitals by a team of doctors and other health professionals. This means that you may need to travel some distance to have your treatment.

Surgery

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 doctors might think that other treatments are needed in addition to, or instead of surgery. Your surgeon or doctor will discuss the most appropriate type of treatment with you and, if you like, with a close relative or friend.

Radiotherapy

Radiotherapy uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. It is usually given by directing radiotherapy rays at the tumour from outside the body – known as external radiotherapy.

The role of radiotherapy in treating chordomas is varied. Radiotherapy may be used to treat chordomas that have not been completely removed with surgery. 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 chordomas include a method called stereotactic radiotherapy. This is a way of giving very precise and accurate radiation.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy the cancer cells. Chemotherapy has not been shown to be very effective in treating chordomas. However, it may be helpful in some people, in controlling a chordoma that has recurred (come back) or has spread elsewhere in the body.

Biological therapies

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. However, more research is needed to find out how effective Glivec is, and at present surgery remains the standard treatment for chordoma.

Research into treatments for cancer is ongoing and advances are being made. Cancer doctors use clinical trials to assess new treatments.

You may be asked to take part in a clinical trial. If this happens, 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 experience many different emotions including anger, resentment, guilt, anxiety 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 illness.

Everyone has their own ways of coping with difficult situations; some people find it helpful to talk to friends or family, while others prefer to seek help from people outside their situation. Others prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is available if you need it.

This section has been compiled using information from a number of reliable sources, including:

• Cancer and Its Management (5th edition). Souhami and Tobias, Oxford Blackwell Scientific Publications, 2005
• The Textbook of Uncommon Cancers (3rd edition). Raghavan et al. Wiley, 2006
• Cancer: Principles and Practice of Oncology (7th edition). DeVita et al. Lippincott Williams and Wilkins, 2005.
• Chordoma of the spine. Panayiotis J et al. Orthopedics. 2004; 27, 12: 1256-1263
• Comprehensive review of intercranial chordoma. Erdem E et al. Radiographics. 2003; 23: 995-1009.

For further references, please see the general bibliography.