Chordoma and proton beam treatment

• What chordomas are
• Causes of chordomas
• Symptoms of chordomas

• Treatment
• New treatments
• Support for people with chordoma

Chordomas are rare cancers. They develop from cells that are formed very early on when a baby’s spine is developing in the womb. The development of the spine starts from a band of fibrous tissue called the notochord. The bones of the spine (vertebrae) then develop around this notochord. When we are born we no longer need the notochord, so it usually disappears. But sometimes some cells remain. It is from these cells that a chordoma can develop. These cells are found at either end of the spinal canal – in the base of the skull and the lower back or tail bone. The tail bone is also called the sacrococcygeal area. Occasionally chordomas are found in other bones, such as the bones of the arms or legs.

Because chordomas are found within the tissue of the spinal canal, doctors sometimes classify them as a bone tumour, and sometimes as a central nervous system tumour.

We don’t know what causes chordomas. They are more common in men than women and are usually diagnosed in people between the ages of 40 and 50. It is a very rare type of cancer, which often means that it’s more difficult for doctors to find out what the causes might be. Doctors in America are researching to see if there may be a hereditary link, although the vast majority of people with chordoma don’t have a family member with this cancer.

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

• Skull chordoma – you may have double vision, headaches, weakness or paralysis in your face
• Chordoma that affects your upper back – you may have hoarseness of your voice or difficulty with swallowing
• Chordoma that affects your lower back – you may have pain, difficulty passing urine or weakness in your legs

It is important that chordomas are treated as soon as possible. This helps reduce the chance of them coming back (recurring). Your treatment will depend on where the chordoma is, and how big it is.

You may have both surgery and radiotherapy. If possible you will have an operation to remove the tumour, or as much of it as possible. Afterwards you might have radiotherapy. The aim of this treatment is to kill off any cells that could have been left behind and so lower the risk of the chordoma coming back. If you can’t have surgery then you will probably still have radiotherapy to shrink the tumour and relieve any symptoms it is causing.

After your treatment has finished, your doctors will want you to come back to hospital regularly so they can see how you are.

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 especially important when you are having treatment to a delicate area of the body, such as the spine.

One of the newer ways of giving radiotherapy uses a different type of beam called a proton beam. Protons collect energy as they slow down and travel through the body. They then release this energy at their target point – the tumour. This means they can deliver a higher dose of radiation straight to the cancer, but only to a very small area around it. So there is less likelihood of damage to nearby healthy tissue, particularly vital tissues and organs behind the tumour, such as the spinal cord or brain stem.

Proton beam radiotherapy machines in the UK are only able to treat cancer of the eye. The machines are not able to treat cancers deeper in the body. Some countries in Europe and the USA are testing and using proton beam radiation for deeper cancers, including chordomas. Currently proton therapy 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 to Switzerland or France, but sometimes to the USA. If you think this treatment may be suitable for you, talk to your own specialist and ask their advice. You may also be able to get advice from one of the organisations we have mentioned below.

In December 2011, the Government confirmed that proton therapy will be made available in the UK.  There will be at least 3 treatment centres, likely to include Manchester, London and Birmingham.  Treatment should be available in the UK from 2014.

The Brain and Spine Foundation provides information and support for people affected by neurological conditions. They have a helpline staffed by neuroscience health professionals, and free booklets on a range of neurological conditions including brain and spinal tumours. Further contact details are in the brain tumour organisations page.

If you have a rare cancer it can be very difficult to meet others facing the same disease. Chordoma Support is an international online support group that has a message board where you can get in touch with other people with chordoma.