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TP53 (Li-Fraumeni Syndrome): Options for Managing Risk

Learn about the screening and prevention guidelines for people with a mutation in their TP53 gene. 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) > Risk Management

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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Risk Management for People with Inherited Mutations

People with mutations have options for managing their increased cancer risk. We recommend that you speak 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 listed below for people with a mutation to manage their cancer risk.

Note that when we use "female" and "women" we are referring to people assigned female at birth.

Guidelines for children	Guidelines for pancreatic cancer
Guidelines for all adults	Guidelines for cancer
Guidelines for breast cancer in women

People with a mutation may also qualify for clinical trials looking for more effective screening or prevention for cancer.

In the News

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. People with inherited mutations in TP53 have Li-Fraumeni syndrome, which is linked to a very high lifetime...

Risk management for children

An inherited mutation can increase the risk for cancer beginning during childhood. Risk management for some cancers begins at a young age.

Beginning Age	Recommendation
infancy	Physical exam including neurologic exam every 6–12 months. Special attention should be paid to look for rare cancers.
infancy	Yearly whole body MRI - including brain MRI.
infancy	Ultrasound to look for adrenal gland cancers every 3-4 months.
Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Breast, Ovarian, Pancreatic, vs. 3 2024.

Risk management for all adults

Beginning Age	Recommendation
18	Physical exam including neurologic exam every 6–12 months. Special attention should be paid to look for rare cancers.
18	Yearly whole body and brain MRI.
18	Yearly skin exam by a dermatologist.
25 (or younger based on family history)	Colonoscopy and upper endoscopy every 2–5 years.
Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Breast, Ovarian, Pancreatic, vs. 3 2024.

Breast cancer risk management for women and people assigned female at birth

Beginning Age	Recommendation	Additional Information
18	Learn to be aware of changes in your breasts.
20 (or earlier based on family history)	Breast exam by doctor every 6-12 months.
20 (or earlier based on family history)	Yearly breast MRI with and without contrast.
30	Yearly mammogram.
75	Have a conversation with your doctor about whether you should continue, stop or change breast screening.
No set age	Discuss the benefits, risks and costs of double mastectomy with your doctor.	Risk-reducing mastectomy lowers breast cancer risk by 90%, but has not been shown to improve survival. Even after double mastectomy, some breast tissue, and therefore cancer risk remains.
Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Breast, Ovarian, Pancreatic, vs. 3 2024.

Pancreatic cancer risk management for people with a family history of pancreatic cancer

Types of pancreatic cancer screening tests

There are two tests that are used to look for pancreatic cancer in high-risk people.

MRCP (Contrast-enhanced magnetic resonance cholangiopancreatography) is a special type of imaging that looks closely at the pancreas, liver, gallbladder, bile duct and pancreatic duct to find abnormalities such as cancer.
EUS (Endoscopic ) involves passing a tiny scope with an attached probe down the esophagus to the stomach. This allows doctors to look closely at the pancreas.

Researchers are testing new approaches to pancreatic cancer screening and prevention through clinical trials.

Participate in research

Blood Markers of Early Pancreas Cancer

Pancreatic cancer guidelines

Beginning Age	Family History	Recommendation
50 (or earlier based on the youngest case of pancreatic cancer in the family)	For TP53 mutation carriers with a first- or second- degree relative with pancreatic cancer.	Discuss the benefits, risks, costs and limitations of screening with your doctor. Screening should include annual MRCP or EUS or both. Screening should be performed in a facility with experience in screening high-risk patients for pancreatic cancer.

cancer risk management

cancer risk

Research suggests that mutations may also increase the risk for cancer. More research is needed to better define the exact risk for people with a mutation.

In addition to the screening guidelines listed below, researchers are testing new approaches to cancer screening and prevention for high-risk people through clinical trials.

Participate in Research

PATROL Study: Prostate Cancer Screening for People AT Genetic Risk FOr Aggressive Disease

cancer screening guidelines

Beginning Age	Recommendation
40	Annual digital rectal exam and Prostate Specific Antigen (PSA) test.
Source: NCCN Guidelines: Genetic/Familial High-Risk Assessment: Breast, Ovarian, Pancreatic, vs. 3 2024