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Cancer Risk
Read about different genes that are linked to hereditary cancer, their associated risks and guidelines for screening, preventing and treating cancers in people with inherited mutations in these genes.
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Hereditary Cancer and Genetic Testing > Hereditary Cancer Genes and Risk > By Gene Mutation > TP53 (Li-Fraumeni syndrome) > Cancer Risk

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Overview
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Cancer Risk
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Risk Management
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Cancer Treatment
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Other Considerations

Cancer risk associated with an inherited mutation

If you have tested positive for a mutation, we recommend that you consult with a genetics expert who can assess your personal and family history of cancer and can help you decide on a plan to manage your risk. Note that when we use the term "men" and "women" we are referring to the sex you were assigned at birth.

People with a mutation are at increased risk for many cancers. The following are the risks for the most common cancers in people with Li-Fraumeni syndrome:

  • breast cancer (about a 60% lifetime risk for women)
  • soft tissue sarcoma (up to 15% for women; 22% for men)
  • osteosarcoma or bone cancer (up to 5% for women; 11% for men)
  • brain tumors (up to 6% for women; 19% for men)
  • pancreatic cancer (5-10% risk)
  • adrenal gland cancer
  • gastric cancer
  • colorectal cancer

Graph of lifetime risk of breast cancer in women with <button x-data class='glossary-tip tt-tp53' x-tooltip='<p>TP53 is a gene found on chromosome 17. It is also sometimes referred as P53. Mutations in TP53&nbsp;are associated with Li-Fraumeni Syndrome. People with Li-Fraumeni&nbsp;Syndrome have an&nbsp;increased the risk for many different types of young-onset cancers. Increased cancer risk associated with Li-Fraumeni syndrome include:</p> <ul> <li>pre-menopausal breast cancer</li> <li>connective tissue cancer</li> <li>bone cancer</li> <li>adrenal cancer</li> <li>pancreatic cancer</li> <li>colon cancer</li> <li>liver cancer</li> <li>childhood cancers</li> <li>brain tumors</li> <li>leukemia</li> </ul> <p>Some people carry TP53 mutations that are associated with a&nbsp;lifetime cancer risk that isn't quite so high; this syndrome is called Li-Fraumeni Like syndrome.</p> <p>Also see Li-Fraumeni&nbsp;Syndrome.</p> ' >TP53</button> mutation     


Childhood cancer risk

Many of the cancers seen in occur particularly early in life, including in children and young adults: 

  • 4% of babies will develop cancer in their first year of life
  • 22% will develop cancer by age 5
  • 41% of children will develop cancer by age 18

Research has shown that plays a role in many childhood cancers, including:

  • 80% of rhabdomyosarcoma with diffuse anaplasia
  • 50% of childhood adrenocortical carcinoma  
  • 40% of choroid plexus carcinoma
  • 40% of low-hypodiploid acute lymphoblastic leukemia
  • 10% of Sonic Hedgehog subtype medulloblastoma
  • 10% of childhood osteosarcoma 
Last updated February 27, 2023

Get Support
Get Support

FORCE offers many peer support programs for people with inherited mutations. 

updated: 08/06/2022

Related Resources
Related Resources

The following resources focus on  mutations. 

 

updated: 03/16/2022