Chordoma

Chordoma is usually slow-growing, so symptoms often take a while to show. Symptoms depend on 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
• problems getting an erection.

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

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

Most of the time, these symptoms are caused by other conditions that are more common than a chordoma. But if you have any of these symptoms get them checked by your GP.

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 may refer you to a specialist. This might be a doctor who specialises in bones, called an orthopaedic doctor. Or it might be a neurologist, who specialises in the nervous system. If they think you may have a chordoma, you should be referred to a hospital with a specialist sarcoma centre for further tests.

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.

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

Chordoma is rare. You will be treated by a team of doctors and other healthcare professionals at a hospital with a specialist sarcoma treatment centre.

Your test results will be discussed by a team of specialist health care professionals. If your tests show a diagnosis of bone cancer, a team of specialist doctors and other professionals will meet to discuss the best possible treatment for you. This is called a multidisciplinary team (MDT).

Your team may also include a:

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

After the MDT meeting, your cancer doctor or specialist nurse will explain the treatment options and possible side effects to you. You will have time to talk to your cancer doctor and nurse about this before you make any treatment decisions. Tell them if you need more information or time to make your decision.

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.

The main treatments for chordoma are surgery and radiotherapy. Occasionally chemotherapy is used.

Surgery is usually the main treatment for chordoma. If possible, the surgeon will remove all of the tumour. If not, it is often possible to remove part of it. This is called debulking surgery. This can help your symptoms by relieving the pressure on the nerves. It can also slow the growth of the tumour.

The surgeon will be careful to try to not damage nearby areas such as the spine, brain or nerves. Sometimes, the surgeon cannot remove the tumour safely. If this is not possible, your cancer doctor may recommend other treatments.

The type of surgery you have and your recovery will depend on:

• the size of the chordoma
• where it is in the body.

If treatment involves removing bone, muscle or nerves, you may find certain movements hard after surgery. Your surgeon will explain the surgery and possible side effects before you make any treatment decisions.

We have more information about surgery for bone cancer.

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

• before surgery to shrink the chordoma
• after surgery, to treat any remaining tumour cells
• as your main treatment, if surgery is not possible
• to help with symptoms such as pain, if the tumour comes back.

Different types of external beam radiotherapy may be used to treat chordoma. They all use different techniques to deliver give high doses of radiotherapy more precisely to the tumour, with lower doses to the healthy tissue nearby. This can help reduce the risk of side effects. The type of radiotherapy used depends on the size and position of the tumour.

The following types of radiotherapy might be used to treat chordoma:

IMRT and VMAT are available in most hospitals. Stereotactic radiotherapy is only available in some hospitals.

Proton beam therapy (PBT) is another type of radiotherapy which is sometimes used to treat chordoma. It uses proton radiation to destroy cancer cells, instead of x-rays. Proton beams stop when they reach the area being treated. This means that PBT causes very little damage to nearby healthy tissue and fewer side effects.

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy is not often used to treat chordoma. It may occasionally be used to control certain types of chordoma that have spread or come back. It may also sometimes be used if surgery is not possible.

Chemotherapy can cause different side effects. This can depend on which chemotherapy drugs you have. We have more information about chemotherapy for bone cancer.

You may be offered some treatments as part of a clinical trial.

We want everyone affected by cancer to feel our information is written for them.

We want our information to be as clear as possible. To do this, we try to:

• use plain English
• explain medical words
• use short sentences
• use illustrations to explain text
• structure the information clearly
• make sure important points are clear.

We use gender-inclusive language and talk to our readers as ‘you’ so that everyone feels included. Where clinically necessary we use the terms ‘men’ and ‘women’ or ‘male’ and ‘female’. For example, we do so when talking about parts of the body or mentioning statistics or research about who is affected.

You can read more about how we produce our information here.