What is chordoma?

Chordoma is a type of sarcoma. Sarcomas are rare cancers that develop in the supporting tissues of the body. Supporting tissues include bone, cartilage, tendons, fat and muscle.

There are 2 main types of sarcoma:

Bone sarcomas can develop in any bone in the body. They can also develop in the soft tissue near bones. Chordoma is a type of bone sarcoma that can develop in the bones of the spine or the bones at the bottom of the skull. Chordomas can develop at any age, but are more common in people aged 40 to 50.

Symptoms of chordoma

Chordomas are usually slow-growing, so symptoms often take a while to show. Symptoms depend on exactly where the tumour is.

If the chordoma starts in the spine, symptoms may include:

  • pain
  • numbness
  • changes in bowel habits, such as constipation
  • problems peeing (passing urine) or controlling the bladder (incontinence)
  • problems walking
  • feeling weak or unsteady
  • in men, problems getting an erection.

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

  • headaches
  • double vision
  • facial pain or numbness
  • changes in hearing
  • problems swallowing
  • feeling dizzy or unsteady.

Causes of chordoma

The exact cause of chordoma is not known. Research into possible causes is happening to find out more about what may cause chordoma. Very rarely, chordoma can be caused by a faulty gene.

Chordoma diagnosis

Tests and diagnosis

You usually start by seeing your GP. They will check the area, and your general health. They may arrange some tests or x-rays.

If your GP is not sure what the problem is, or thinks you may have a bone cancer, they will refer you to a specialist hospital for tests.

Tests to diagnose bone cancer, such as bone biopsies, are done by a specialist team.

Your team may include:

  • a specialist surgeon who operates on the spine and brain
  • a specialist surgeon who operates on the bottom of the skull
  • a neurologist – a doctor who treats illnesses of the brain and nervous system
  • a neuro-otologist – an ear, nose and throat (ENT) doctor who treats illnesses affecting the bottom of the skull and nearby nerves
  • a clinical oncologist – a doctor who treats cancer with radiotherapy, chemotherapy and other drugs
  • a specialist nurse – who gives information and support to people with primary bone cancer.

The doctor at the hospital will ask you about your general health and any previous medical problems. They will check the area of bone for any swelling or pain. You will have a blood test to check your general health.

Your cancer doctor may arrange some of the following tests to diagnose chordoma. These tests also check if the cancer has started to spread.

  • MRI scan

    An MRI scan uses magnetism to build up a detailed picture of areas of your body.

  • Bone biopsy

    A bone biopsy means the doctor takes a sample of cells from the bone to be checked for cancer under the microscope.

  • CT scan

    Most patients with bone cancer will have a CT scan of their lungs. They might also have a CT scan of the affected bone. 

Waiting for test results can be a difficult time, we have more information that can help.

Treatment of chordoma

Chordomas are rare, so they are treated by a team of doctors and other healthcare professionals at a specialist hospital. This means you may have to travel to have treatment.

The type of treatment you have depends on:

  • your age
  • the position and size of the cancer
  • if it has spread to other parts of the body
  • your general health.

Your cancer doctor and specialist nurse will explain your treatment and what will happen. They will talk to you about the benefits and disadvantages of treatment. They will also explain the risks and side effects. You may be offered some treatments as part of a clinical trial.

The treatment may be used to cure the chordoma or to control it. You may be given a choice of treatment options. You will have time to talk to your cancer doctor and nurse about this before you make any treatment decisions. Tell your cancer doctor if you need more information or time.

The main treatments for chordoma are surgery and radiotherapy. Sometimes chemotherapy or other drugs are used.

Surgery for chordoma

The type of surgery you have and your recovery will depend on the size and location of the chordoma. Your surgeon will talk to you about the different types of surgery before you make any treatment decisions. We have general information about surgery for bone cancer.

Surgery is used to try to remove the chordoma without damaging nearby areas. These include the spine, brain or nerves. If possible, the surgeon will remove all of the tumour. If not, it is often possible to remove part of it (debulking surgery). This can help your symptoms by relieving the pressure on the nerves. It can also slow the growth of the tumour.

Sometimes, the surgeon cannot remove the tumour safely. If this happens, your cancer doctor may recommend other treatments.

Radiotherapy for chordoma

Radiotherapy uses high-energy rays to destroy the cancer cells. We have more information about radiotherapy for bone cancer. You may have this:

  • after surgery, to treat any remaining tumour cells
  • as your main treatment if surgery is not possible
  • with chemotherapy if surgery is not possible
  • if the tumour comes back.

Radiotherapy is also sometimes used to help with symptoms, such as pain.

Different types of radiotherapy may be used to treat chordoma. These include:

  • stereotactic radiotherapy
  • intensity-modulated radiotherapy (IMRT)
  • VMAT or ARC therapy.

These types give high doses of radiotherapy directly to the chordoma.

Proton beam therapy (PBT) is another type of radiotherapy sometimes used to treat chordoma. This treatment is not yet widely available in the UK, so you may need to go to a different country to have it. Your cancer doctor can give you more information if PBT is right for you.

Chemotherapy for chordoma

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy is sometimes used to try to control chordoma that has come back or has spread. It may also be used if surgery is not possible.

Chemotherapy can cause different side effects. This can depend on which chemotherapy drugs you have. Your cancer doctor will tell you more about treatments that may be useful for you.We have more information about chemotherapy for bone cancer.

Targeted therapies for chordoma

Targeted therapies change the way cancer cells grow. This can control or shrink a cancer. You may be offered this type of treatment as part of a clinical trial.

After chordoma treatment


After you finish your treatment, you will have regular check-ups and x-rays. These will continue for a few years. Tell your cancer doctor as soon as possible if you have any problems, or notice any new symptoms in between your regular follow-up appointments.

Possible late side effects

A small number of people may develop late side effects from the treatment, sometimes many years later. Late effects may include:

If treatment involves removing bones and muscle, you may find day-to-day tasks hard. How hard you find things will depend on where in the body the cancer started. It will also depend on whether you had limb-sparing surgery or an amputation.

Your cancer doctor or specialist nurse will explain more about any possible late side effects.

Getting support

You may have many different emotions, including feeling angry, sad, and scared. You may cry a lot or you might find it hard to relax. You may have problems sleeping. These are normal reactions, but they can be hard to cope with. It is important to remember there is lots of support to help you.

How much support you need is different for everyone. It may depend on the treatment and any side effects you have. Your cancer doctor will tell you about any possible side effects and how to deal with them before you start treatment.

We have more information about coping with difficult feelings and the support available. You may also find the following organisations helpful.

Organisations such as Sarcoma UK and Bone Cancer Research Trust can provide information and support. Cancer52 represents many charities and works to improve the quality of life for people with rarer types of cancer.

Macmillan is also here to support you. If you would like to talk, you can:

Reviewed: 31 March 2019
Reviewed: 31/03/2019
Next review: 30 September 2021
Next review: 30/09/2021

This content is currently being reviewed. New information will be coming soon.