Chordoma is a rare type of primary bone cancer. It can develop in the bones of the spine or the base of the skull. It is usually slow-growing.

Symptoms can vary depending on the position of the chordoma. You will have tests including an MRI or CT scan and often a biopsy.

Treatment depends on your age and general health, and the position and size of the chordoma. Usually, you have surgery to remove the chordoma or as much of it as possible. Radiotherapy may be used after surgery, or as the main treatment if surgery is not possible. Sometimes, anti-cancer drugs such as chemotherapy or targeted therapies are used.

Your doctor will explain the best treatment for you. They will tell you about any risks or side effects of treatment. You may have to travel to a hospital that offers specialist treatment for chordoma.

After treatment finishes, you will have regular check-ups.

What is chordoma?

Chordoma is a rare type of primary bone cancer. It can develop in the bones of the spine or the bones at the base of the skull.


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This type of cancer starts in the notochord. The notochord forms the spine in a developing baby in the womb. By the time a baby is born, the bones of the spine have grown and replaced most of the notochord. Only small amounts of the notochord are left.

Chordomas tend to be slow-growing and do not often spread to other parts of the body. If they do spread, the most commonly affected places are the lungs, bone, skin and brain.

Chordomas can happen at any age, but mainly affect people aged 50 to 60.

Causes and risk factors of chordoma

The exact cause of chordoma is unknown. Research into possible causes is being done. Very rarely, chordoma runs in families.

Signs and symptoms of chordoma

Chordomas usually grow slowly, so symptoms often develop gradually. Symptoms depend on exactly where the tumour is in the spine or skull.

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

  • pain
  • numbness
  • changes in bowel habits, such as constipation
  • problems passing urine or poor bladder control
  • problems walking
  • feeling weak or unsteady.

Men may also have problems getting an erection.

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

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

Diagnosing chordoma

You usually start by seeing your family doctor (GP). They will examine you and may arrange tests or x-rays. If your GP thinks your symptoms might be caused by a chordoma, they will refer you to a specialist team at a hospital. Your nearest hospital may not have a specialist team for primary bone or skull base tumours. You may have to travel to a hospital that provides this.

Your team may include:

  • a specialist surgeon who operates on the spine and brain
  • a specialist surgeon who operates on the skull base
  • 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 skull base 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 tumours.

At the hospital, your doctor will examine you and explain what other tests you need. If you haven’t already had a scan, you will have an MRI or a CT scan. You will usually also have a biopsy. These tests give your team as much information as possible about the type, position and size of the tumour, so they can plan your treatment.

MRI (magnetic resonance imaging) scan

This test uses magnetism to build up a detailed picture of areas of your body. Before the scan, you’ll be asked to remove any metal belongings including jewellery. Some people are given an injection of dye into a vein in the arm, which doesn’t usually cause discomfort. This is called a contrast medium and can help the images from the scan to show up more clearly.

During the test you lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It’s painless but can be slightly uncomfortable, and some people feel a bit claustrophobic. It’s also noisy, but you’ll be given earplugs or headphones. You can hear, and speak to, the person operating the scanner. We have more detailed information about having an MRI scan.

CT (computerised tomography) scan

A CT scan uses x-rays to build a three-dimensional picture of the inside of the body. You may be given either a drink or an injection of dye. This is to make certain areas of the body show up more clearly. This scan takes around 30 minutes and is painless. We have more detailed information about having a CT scan.


During a biopsy, a small piece of tissue or a sample of cells is removed from the tumour. This is then examined under a microscope to find out more about the tumour. Sometimes, a scan or ultrasound is used to help the doctor guide a biopsy needle to the exact area.

The biopsy may involve an operation, so you may have to stay in hospital for a few days. How it is done will depend on the position of the tumour. Your doctor will tell you if you need to have a biopsy and explain exactly what your operation involves.

Treating chordoma

Your treatment will depend on:

  • your age
  • your general health
  • the size and position of the tumour.

Chordomas are rare, so there are only a few hospital teams in the UK who specialise in treating them. This means you may need to travel for treatment.

Your specialist doctor and nurse will explain the aims of your treatment and what it involves. They will talk to you about the benefits and disadvantages of treatment. They will also explain the risks and side effects.

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

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


The aim of surgery is to remove the chordoma without damaging nearby areas such as the spine, skull or nerves. If possible, the surgeon will remove the tumour completely. If not, it is often possible to remove part of it (debulking surgery). This can improve your symptoms by relieving the pressure on the nerves. It can also slow down the progress of the tumour.

The type of surgery you have, and your recovery, will depend on the size and position of the tumour. Your surgeon will discuss your operation with you in detail so that you know exactly what is involved.

Sometimes, the surgeon cannot remove the whole tumour safely. In this situation, your doctor may recommend other treatments after, or instead of, surgery.


Radiotherapy uses high-energy rays to destroy the cancer cells. You may have this:

  • after surgery to treat any remaining tumour cells
  • as your main treatment if surgery isn’t possible
  • with chemotherapy if surgery isn’t possible
  • if the tumour comes back.

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

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

  • stereotactic radiotherapy
  • image-guided radiotherapy (IGRT)
  • intensity-modulated radiotherapy (IMRT).

These types give high doses of radiotherapy very accurately to the chordoma.

Proton beam therapy is another type of radiotherapy sometimes used to treat chordoma. This treatment is not yet available to treat chordoma in the UK. But in some situations, the NHS may arrange for you to have it overseas. Proton beam therapy will hopefully be available in the UK from 2018. We have more information on the different types of radiotherapy

Radiotherapy can cause side effects. Your side effects may depend on the type of radiotherapy and the position of the chordoma. Side effects of radiotherapy may include:

  • skin reactions
  • feeling sick (nausea)
  • feeling tired (fatigue).

Some people find the side effects temporarily get worse after their treatment has finished. You may feel low or worry your treatment isn’t working. But this is usually a reaction to the radiotherapy. Let your doctor or nurse know if you have side effects or they get worse, so they can help.


Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy may sometimes be used to try to control a chordoma that has come back or has spread. It may also be used if surgery isn’t possible.

Chemotherapy can cause different side effects. These vary depending on the drugs you have. Your cancer doctor will tell you more about treatments that may be useful for you.

Some side effects are more common than others, but you won’t have them all. They can be reduced and controlled with drugs. Your doctor or nurse can tell you how to manage them. The most common ones include:

  • increased risk of infection
  • anaemia (reduced number of blood cells)
  • hair loss or thinning
  • feeling sick (nausea) or vomiting
  • mouth ulcers
  • tiredness (fatigue).

Most side effects are short term and will improve gradually when the treatment is over.

Targeted therapies

These drugs target certain features of the cancer cells but have little effect on most other cells. They interfere with how cancer cells grow and survive. This can control or shrink a cancer for a time.

Several targeted therapy drugs, including imatinib (Glivec®) and sunitinib (Sutent®), are being tested as treatments for chordoma. You may be offered this type of treatment as part of a clinical trial.

Different drugs have different side effects. Your doctor will explain what side effects are likely in your situation. We have more information about targeted therapies.

Clinical trials

You may be asked to take part in a type of research called clinical trials. Research is used to find out about newer treatments for chordoma such as imatinib (Glivec®) or sunitinib (Sutent®). It may also be used to find out more about chordoma by looking at tumour samples from lots of patients. 

Your doctor will discuss any trial with you so that you understand what it means to take part. You may decide not to take part, or to withdraw from a trial at any stage. In this case, you will still receive the best standard treatment available.


After your treatment has finished, you will have regular check-ups and possibly scans. These may continue for several years. This is a good time to discuss any worries or problems you have with your doctor. If you notice any new symptoms between your check-ups, let your doctor know as soon as possible.

Many people find they get very anxious before follow-up appointments. This is natural. You might find it helps to talk to family or friends. Or you may want to contact us for support.