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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.

Hereditary Cancer and Genetic Testing > Hereditary Cancer Genes and Risk > Risk Management and Treatment by Mutation > TP53 (Li-Fraumeni syndrome) > Cancer Risk

TP53: The Basics TP53 (Li-Fraumeni Syndrome): Cancer Risks TP53 (Li-Fraumeni Syndrome): Options for Managing Risk TP53: Options for Cancer Treatment TP53 (Li-Fraumeni Syndrome): Other Considerations

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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 STK11 Mutation	Lifetime Risk in General Population
Breast cancer in women (people assigned female at birth)	More than 60%	12.5%
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.

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: