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Chordomas

Chordomas develop from cells formed early on when a baby's spine is developing in the womb. Find out more about diagnosis and treatment.

What chordomas are

Chordomas are a very rare, slow growing type of bone cancer. 

Chordomas develop from the notochord, which forms the early spinal tissue in a baby developing in the womb. After about 6 months, this tissue is replaced by bone. But sometimes small areas of notochord may remain.

About 2 out of 5 chordomas (40%) grow in the skull or the bones in the middle of the face. The rest develop in the bones of the spine.

Because chordomas are found within the tissue of the spinal canal they are sometimes classified as bone tumours and sometimes central nervous system tumours.

Causes of chordomas

Because chordomas are a very rare type of cancer it is more difficult for doctors to find out what the causes might be. They are more common in men than women and are usually diagnosed between the ages of 40 and 50.

Researchers are interested in finding out if there is a genetic link, although the majority of people with a chordoma don't have a family member with this cancer.

Symptoms

Chordomas tend to be slow growing. People tend to have symptoms for many months before they are diagnosed. The symptoms you have depend on where the chordoma is. 

Skull chordoma

You might have:

  • double vision
  • headaches
  • weakness
  • paralysis in your face

Chordoma that affects your upper back

You might have hoarseness of your voice or difficulty with swallowing.

Chordoma that affects your lower back

You might have:

  • pain
  • difficulty passing urine
  • weakness in your legs

Diagnosing chordomas

Your doctor will examine you and you may have blood tests to check your general health. Other tests might include:

  • a CT scan
  • a MRI scan
  • a biopsy

Treatment

Your treatment depends on where the chordoma is and how big it is. It's important your chordoma is treated as soon as possible. This helps reduce the chance of it coming back (recurring).

You might have an operation to remove your tumour or as much of it as possible. Afterwards you might have radiotherapy. The aim of radiotherapy is to kill off any cells that have been left behind and lower the risk of the chordoma coming back.

If you can't have surgery, you might have radiotherapy to shrink the tumour and relieve any symptoms.

Follow up

You will have regular check ups once you finish your treatment. Your doctor will examine you and ask about your general health.

This is your chance to ask questions and to tell your doctor if anything is worrying you.

How often you have check ups depends on your individual situation.

Research and clinical trials

There may be fewer clinical trials for rare types of cancer than for more common types.

It is hard to organise and run trials for rare cancers. Getting enough patients is critical to the success of a trial. The results won't be strong enough to prove that one type of treatment is better than another if the trial is too small.

The International Rare Cancers Initiative (IRCI) aims to develop more research into new treatments for rare cancers. They are designing trials that involve several countries so that more people will be available to enter trials.

Proton beam therapy

Radiotherapy can cause side effects. Doctors are looking at new ways of giving radiotherapy to reduce these side effects, while still treating the cancer. This is important when you are having treatment to a delicate part of the body.

One of the newer ways of giving radiotherapy uses a different type of beam called a proton beam. This means doctors can deliver a higher dose of radiation straight to the cancer and less to the healthy tissue around it.

Some countries in Europe and the USA are testing and using proton beam radiation for some cancers, including chordomas. At the moment proton beam radiotherapy for chordomas is not available in the UK. If this treatment is suitable for you the NHS pays to send you for treatment abroad, usually the USA.

Talk to your specialist if you think this treatment might be suitable for you.

Coping

Coping with a diagnosis of cancer can be difficult, both practically and emotionally. It can be especially difficult if you have a rare cancer. Being well informed about your cancer and its treatment can make it easier to make decisions and cope with what happens.

Talking to other people who have the same thing can help. But it can be hard to find people who have had a rare type of cancer.

Support

Cancer Research UK’s discussion forum is called Cancer Chat. It is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.

The Rare Cancer Alliance and the Rare Cancer Support Forum both offer support and information to people who have rare cancers.

The Chordoma Foundation is an international online support group that has a message board where you can get in touch with other people with chordoma.

The Brain and Spine Foundation provides information and support for people affected by neurological conditions. They have a helpline staffed by neuroscience health professionals. 

You could call the Cancer Research UK nurses free on 0808 800 4040 to talk about proton beam therapy. They are available 9am to 5pm, Monday to Friday.
Last reviewed: 
01 Sep 2014
  • National Proton Beam Therapy Service Development Programme
    Department of Health, 2012

  • Principles and practice of oncology (9th edition)
    De Vita, V.T., Hellman, S. and Rosenberg S.A.
    Lippincott, Williams and Wilkins, 2011

  • The Textbook of Uncommon Cancers (4th edition)
    Raghavan et al
    Wiley, 2012

Information and help

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