Chordoma is a type of primary bone cancer (also called bone 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:
Chordoma is a rare type of bone sarcoma. It can develop in the bones of the spine or the bottom of the skull. Chordoma can develop at any age but is more common in people aged 40 to 50.
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:
- 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:
- 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.
Before birth, cells called notochord cells help to build the spine of an embryo. Some of the notochord cells remain in the bones of the spine and bottom of the skull after birth. Very rarely, chordoma tumours might develop from these cells. We do not know what causes the cells to become cancerous. Research continues to find out more about the possible causes of chordoma.
Rarely, chordoma can be caused by a faulty gene. But this is not the cause of most chordomas.
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.
The results of your tests give your cancer doctor information about the stage and grade of the chordoma.
The stage refers to the size of the chordoma and whether it has spread outside the bone.
The grade of the cancer is how the cancer cells look under the microscope. This gives an idea of how quickly a cancer may grow and develop.
The most common grading system for primary bone cancer uses the following 3 grades:
- Grade 1 - cancer cells are low-grade and look like normal bone cells. They are usually slow-growing and less likely to spread.
- Grade 2 - cancer cells are high-grade and look abnormal. The cells are likely to grow more quickly and are more likely to spread.
- Grade 3 - cancer cells are high-grade and look more abnormal than grade 2. This means the cancer is more likely to come back (recur) or spread to other parts of the body.
Chordoma is usually low-grade or slow-growing. There are a small number of chordomas that are high-grade and grow more quickly.
We have more information about staging and grading bone cancer.
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 for chordoma
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 for chordoma
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:
Intensity-modulated radiotherapy (IMRT)
IMRT shapes the radiotherapy beams and allows different parts of the treatment area to have different doses of radiotherapy.
Volumetric-modulated arc radiotherapy (VMAT)
This is a newer way of giving IMRT. During treatment, the radiotherapy machine moves around you and reshapes the beam. It is sometimes called RapidArc®.
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 for chordoma
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.
You may be offered some treatments as part of a clinical trial.
Possible late effects
A small number of people have side effects that continue after treatment. This will vary depending on the size and position of the tumour and the treatment. Sometimes side effects develop after treatment and some may happen many years later. Late effects for chordoma may include:
- nerve damage
- bone fractures.
You may have rehabilitation to help you recover and manage any side effects.
Your cancer doctor or specialist nurse will explain more about any possible late effects and what can help to manage them. Always tell them if side effects are not improving.
Sex and fertility
Cancer and its treatment can sometimes affect your sex life. There are ways to help your sexual well-being and to manage any problems.
Treatment for sarcoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:
After finishing treatment, you may still be coping with difficult feelings. Talking to your family and friends or health professionals about how you feel can help to support your well-being.
Macmillan is also here to support you. If you would like to talk, you can:
Below is a sample of the sources used in our chordoma information. If you would like more information about the sources we use, please contact us at email@example.com
European Society for Medical Oncology ESMO. Clinical Practice Guideline for diagnosis, treatment and follow-up. S.J. Strauss et al. December 2021. Available from Bone sarcomas: ESMO–EURACAN–GENTURIS–ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up - Annals of Oncology (accessed May 2023)
Journal of Clinical Medicine. Chordoma - Current understanding and modern treatment paradigms. Sean M. Barber. March 2021. Available from Chordoma—Current Understanding and Modern Treatment Paradigms - PMC (nih.gov) (accessed May 2023)
National Institute for Health and Care Excellence (NICE). Bone and soft tissue sarcoma - recognition and referral: Diagnosis of bone and soft tissue sarcoma. Last revised August 2020. Available from https://cks.nice.org.uk/topics/bone-soft-tissue-sarcoma-recognition-referral/diagnosis/ (accessed May 2023)
Journal of Pathology and Translational Medicine. What’s new in soft tissue and bone pathology 2022–updates from the WHO classification 5th edition. Erica Y. Kao 1 and Jose G. Mantilla 2. November 2022. Available from What’s new in soft tissue and bone pathology 2022–updates from the WHO classification 5th edition - PMC (nih.gov) (accessed May 2023)
This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by senior medical editor Fiona Cowie, Consultant Clinical Oncologist.
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