Causes of chondrosarcoma

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This information has been written for patients, their families and friends, and the general public to help them understand more about chondrosarcoma: what it is and the different types. This information is produced in accordance with BCRT's information policy.

What causes chondrosarcoma?

There has been a lot of research into possible causes of chondrosarcoma but the underlying cause remains unknown in most cases.

Chondrosarcoma is not contagious. It cannot be passed on to another person by exposure to a chondrosarcoma patient.

It is known that cells contain important information in their chromosomes called genes, which help them to divide and grow normally. Damage to these genes in one single cell can cause the cell to behave differently and grow abnormally, which can then lead to development of cancer.

In chondrosarcoma, there is damage to the cell's genetic information such as abnormalities of genes (mutations) called tumour suppressor genes and oncogenes as well as genes which control the copying of the cell’s DNA. We do not know why the damage occurs in most cases. In some young patients we know that chemotherapy or radiotherapy for another tumour can damage the DNA enough to cause chondrosarcoma

(Lin et al., 2010, Lor Randal and Hunt, 2010, Kleihues et al., 2006, Lewis and Ries,).

Some recent research has identified a pair of genes that are frequently damaged in chondrosarcoma tumours. Researchers looked in cells from many different chondrosarcoma tumours and found that two genes, called IDH1 and IDH2, were damaged (mutated) in many tumours. These genes are also found to be damaged in some other cancer types. We don’t know why these genes get damaged but we do know that the damage is not inherited (Amary et al 2011).

A small proportion of patients are known to have inherited damaged genes from their parents. This is known as a genetic predisposition, which means they have an increased risk of developing different types of cancer, including chondrosarcoma. Some examples of this are in the section about 'risk factors for chondrosarcoma.'

Doctors and scientists all over the World are involved in research to try to understand the difference between normal bone cells and chondrosarcoma cells. This may enable them to find treatments that can target the abnormal chondrosarcoma cells rather than normal cells in the body (see How is Chondrosarcoma Treated? section for more details on how studying the biology of chondrosarcoma can lead to new targets for therapies).

Chondrosarcoma risk factors

Although doctors do not yet fully understand what causes chondrosarcoma, there are several factors that put people at a higher risk. These factors are known as chondrosarcoma risk factors. A risk factor is something that can increase the chance of getting an illness.

Risk factors

  • Gender:
    Males have a slightly higher risk compared to females.
  • Age:
    Most chondrosarcomas develop in people over the age of 40.
  • Underlying bone diseases:
    Bones which already have something wrong with them (underlying bone abnormalities), for example Paget’s Disease and Ollier's Disease, are at a higher risk of chondrosarcoma. These diseases are very rare.
    • Paget's Disease is a disease that makes bones painful and encourages bone cells to divide more rapidly.
    • Ollier's Disease is a condition in which benign tumours in the bones cause the affected bones to swell. The condition usually presents before age 10. Patients with Ollier’s Disease have around a 20-25% chance of developing chondrosarcoma. Many Ollier’s Disease patients have mutations in IDH1 or IDH2 genes.
    • Maffucci Syndrome is a rare disorder that gives people with this condition a 20-25% risk of developing chondrosarcoma. Many Maffucci Syndrome patients have mutations in IDH1 or IDH2 genes.

(Amery et al 2011)

Inherited risk factors

These are risk factors that people inherit from their parents and have from birth (hereditary). They can be passed on from one or both parents. The risk is passed to the child through the parent's genes. So, just as children inherit features such as hair or eye colour from their parents, a very small number can inherit a risk that will increase their chance of getting chondrosarcoma.

Certain hereditary conditions may make people more susceptible to chondrosarcomas.

  • Multiple Hereditary Exostoses (hereditary skeletal disorder), patients with this condition have a 0.6% to 2.8% risk of developing chondrosarcoma.

People with these conditions are at a higher risk because they can develop benign bone tumours, which can become malignant.

  • Wilm’s tumour (Kidney tumour). In extremely rare cases, chondrosarcomas have been seen as secondary tumours from a primary Wilm’s tumour.

Environmental risk factors

  • Treatment by Radiation or chemotherapy for a pre-existing condition. People who have received radiotherapy for cancer before have a slightly higher chance of developing chondrosarcoma.

(Lin et al., 2010, Lor Randal and Hunt, 2010, Kleihues et al., 2006, Lewis and Ries,)

Information about what causes a disease is called 'aetiology' (EE-tee-OL-loh-jee).


The authors and reviewers of this information are committed to producing reliable, accurate and up to date content reflecting the best available research evidence, and best clinical practice. We aim to provide unbiased information free from any commercial conflicts of interest. This article is for information only and should not be used for the diagnosis or treatment of medical conditions. BCRT can answer questions about primary bone cancers, including treatments and research but we are unable to offer specific advice about individual patients. If you are worried about any symptoms please consult your doctor.

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BCRT shall hold responsibility for the accuracy of the information they publish and neither the Scheme Operator nor the Scheme Owner shall have any responsibility whatsoever for costs, losses or direct or indirect damages or costs arising from inaccuracy of information or omissions in information published on the website on behalf of BCRT.

Version 2 produced January 2013
Information will be reviewed in January 2015

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