Chordomas develop from cells formed early on when a baby's spine is developing in the womb.
What are chordomas?
Chordomas are a very rare, slow growing type of bone cancer.
Chordomas grow from the notochord, which forms the early spinal tissue in a baby developing in the womb. During development, the notochord gets replaced by bone. But sometimes, small areas of notochord may remain in adults.
About 35 out of 100 chordomas (35%) grow in the base of the skull and 50 out of 100 (50%) in the bone (sacrum) between the hip bones. The rest develop in the bones of the spine.
Causes of chordomas
Chordomas are a very rare type of cancer, so 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 60.
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.
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.
You might have:
- double vision
- paralysis in your face
Chordoma that affects you neck
You might have hoarseness of your voice or difficulty with swallowing.
Chordoma that affects your lower back
You might have:
- difficulty passing urine
- weakness in your legs
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
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.
You have regular appointments with your doctor or nurse after treatment finishes. Your doctor examines you at each appointment. They ask how you are feeling, whether you have had any symptoms or side effects, and if you are worried about anything. You might also have MRI scans on some visits.
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.
Go to Cancer Research UK’s clinical trials database if you are looking for a trial for chordoma in the UK. You need to talk to your specialist if there are any trials that you think you might be able to take part in.
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.
Up until recently everyone needing high energy proton beam therapy had to go abroad for treatment.
In 2009, the UK government made the decision to set up a National NHS Proton Beam Therapy Service. Currently there is one centre at The Christie NHS Foundation Trust in Manchester, offering high energy proton beam treatment for anyone needing it in the UK. In 2021, another centre will open at University College London Hospital (UCLH).
It will take a while for both these centres to be fully up and running. So some people will still need to travel abroad for treatment for the next couple of years.
Proton beam therapy is also available in private treatment centres in the UK. So you would have to pay for this treatment or use private insurance if you have it.
A small number of people in South Wales may have proton beam therapy on the NHS at the Rutherford Cancer Centre. This is a private treatment centre in Newport. You have the PBT there and the rest of your care is at your usual cancer treatment centre.
Talk to your specialist if you think this treatment might be suitable for you.
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.
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.