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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.

Experts at the National Comprehensive Cancer Network (NCCN) created guidelines which estimate the cancer risks for people with mutations. These cancer risk estimates, are based on the latest research and include the following:

Risks for adults

Cancer Type	Lifetime Risk with Mutation	Lifetime Risk in General Population
Breast cancer in women	More than 60%	12.5%
10-year risk for 2nd breast cancer diagnosis in women diagnosed with breast cancer	18-49%
cancer	25-50%	12%
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/Familial High-Risk Assessment: Breast, Ovarian, Pancreatic, , v. 1, 2026; Mai PL, Best AF, et. al. Risks of first and subsequent cancers among mutation carriers in the National Cancer Institute 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: