Li-Fraumeni Syndrome (LFS) is a rare genetic condition that makes people more likely to develop cancer at a young age. It is caused by a change (mutation) in a gene called TP53, which normally helps prevent cancer. ACC Support UK does not provide detailed information on or support for those with LFS, but we hope that the following is useful in answering some of your questions, and in directing you to other organisations that support LFS.
How does LFS Cause Cancer?
- The TP53 gene controls how cells grow and repair damage.
- When this gene is mutated, it doesn’t work properly, so cells can grow out of control and turn into cancer.
Who can get LFS?
- LFS is inherited, meaning it is passed down from parents to children.
- A person with LFS has a 50% chance of passing it to their children.
What Cancers are Linked to LFS?
People with LFS have a high risk of getting different cancers, often at a young age. These include:
- Brain tumours
- Bone and muscle cancers (sarcomas)
- Breast cancer
- Adrenocortical carcinoma (ACC) (a rare cancer of the adrenal gland)
- Leukaemia and lymphoma (blood cancers)
How is LFS Diagnosed?
Doctors can test for TP53 mutations with a genetic test, usually a simple blood test.
Can LFS be Treated?
There is no cure for LFS, but people with it can:
- Have regular check-ups and scans to catch cancer early.
- Avoid radiation therapy, as it can increase cancer risk.
- Consider genetic counselling to understand risks for future children
Since people with LFS have a higher chance of cancer, early detection and monitoring are very important.
Relevant Useful Organisations
- Li-Fraumeni Syndrome Association (USA)
- Living LFS (USA)
- The George Pantziarka TP53 Trust (UK)