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TP53 (Li-Fraumeni Syndrome): Cancer Risks

Learn about the cancer risks associated with a TP53 mutation. Stay up to date by signing up for our community newsletter.
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Cancer Risks Associated with Inherited Mutations
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If you have tested positive for an inherited mutation, we recommend speaking with a genetics expert who can look at your personal and family history of cancer, and can help you decide on a plan for managing your risk. Note that when we use "women" we are referring to people assigned female at birth.

cancer risks include the following:

Risks for adults

Cancer Type
Lifetime Risk with TP53 Mutation
Lifetime Risk in General Population

Breast cancer in women (people assigned female at birth)

More than 60%

12.5%

10-year risk for 2nd breast cancer diagnosis in women diagnosed with breast cancer

18-49%

Soft tissue sarcoma in men
22%

less than 1%

Soft tissue sarcoma in women

15%

less than 1%
Bone sarcoma in men
11%

less than 1%

Bone sarcoma in women
5%
less than 1%
Brain cancer in men
19%
less than 1%
Brain cancer in women
6%

less than 1%

Colorectal cancer
20%
4.1%
Pancreatic cancer
5%
1.5%

Source: NCCN: Genetic/Familiar High-Risk Assessment: Breast, Ovarian, Pancreatic Version 3.2024; Mai PL, Best AF, et. al. Risks of first and subsequent cancers among TP53 mutation carriers in the National Cancer Institute Li-Fraumeni syndrome cohort. Cancer. 2016 Dec 1;122(23):3673-3681. doi: 10.1002/cncr.30248. Epub 2016 Aug 6. PMID: 27496084; PMCID: PMC5115949.


Risks for children

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 

Factors that affect risk

 

It is important to note that cancer risks are estimates over the course of a person's lifetime. Your lifetime risk and risk over the next five years will vary depending on:

  • current age
  • sex assigned at birth
  • specific mutation
  • personal and family health history
  • diet, exercise, lifestyle and other factors

Visit our partner

Li-Fraumeni Syndrome Association

LFSA provides information, advocacy and resources for individuals and families with Li-Fraumeni syndrome.

Participate in research

Personal story

Living a full life with Li-Fraumeni syndrome

Personal Story : Living a full life with Li-Fraumeni syndrome

This XRAY review is about a five-time cancer survivor who has an inherited mutation in the TP53 gene....

Last updated September 04, 2024