Chordomas

Chordomas are a very rare, slow growing type of primary bone cancer. Chordomas develop from cells formed early on when a baby's spine is developing in the womb. These are called the notochord cells.

During development, the notochord cells get replaced by bone. But sometimes, small areas of notochord cells may remain in adults.

About 30 out of 100 chordomas (about 30%) grow in the base of the skull. Around 50 out of 100 (around 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.

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 neck

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).

Surgery

You might have an operation to remove your tumour. Or you might have surgery to remove as much of the tumour as possible. This is called debulking surgery.

Radiotherapy

You might have a type of radiotherapy called external radiotherapy. It directs radiation beams at the cancer cells to destroy it from outside of the body.

After surgery some people go onto have radiotherapy. The aim of radiotherapy after surgery is to kill off any cancer cells that have been left behind. This is to lower the risk of the chordoma coming back.

If you can't have surgery, you might have external radiotherapy as your main treatment. The aim is to shrink the tumour and relieve any symptoms you might be getting.

Some people might have a type of external radiotherapy called proton beam therapy .

Follow up

You have regular appointments with your healthcare team after treatment finishes.

You usually have an examination at each appointment. They also ask:

• how you are feeling
• whether you have had any symptoms or side effects
• if you are worried about anything

You also have regular MRI scans.

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

Research and clinical trials

Researchers are looking into chordomas and want to find out:

• how they might develop
• if there are newer treatments

There may be fewer clinical trials for rare types of cancer than for more common types. This is because 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. Talk to your specialist if there are any clinical trials that you think you might be able to take part in.

Coping and support

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.

Other organisations

Chordoma UK

Chordoma UK provides information and support through an online community. They also fundraise to support research into chordoma.

Chordoma Foundation

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.

Bone Cancer Research Trust

This organisation provides information, support and counselling for people with primary bone cancer and their families. They promote research into the causes and treatment of primary bone cancer, in particular osteosarcoma and Ewing sarcoma. 

Telephone: 0113 258 5934

The Rare Cancer Alliance

The Rare Cancer Alliance offer support and information to people affected by rare cancers. It has a forum where you might be able to meet others with the same cancer as you. 

Rare Cancer Support Forum

The Rare Cancer Support Forum has several message boards so people with less common or rarer cancers can get in touch with each other. Friends and families of people with rarer cancers can also use the message boards to share their experiences, give support or get advice from people in similar situations.

Brain and Spine Foundation 

This organisation provides support and information for people with neurological conditions and their relatives. It also produces free booklets on these conditions, including brain and spinal tumours.

Helpline: 0808 808 1000
Email: helpline@brainandspine.org.uk