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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 Genes and Risk > By Gene Mutation > TP53 > Cancer Risk

1
Overview
2
Cancer Risk
3
Risk Management
4
Cancer Treatment
5
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 determine the best risk management plan.

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 

find-support find-support

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

updated: 03/12/2022

related-resources related-resources

The following resources focus on  mutations. 

 

updated: 03/16/2022

Last updated January 23, 2022