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Li-Fraumeni Syndrome

Li-Fraumeni Syndrome - Symptoms

What are the cancer risks associated with LFS?

Cancer risks for individuals aged 20 and under with a faulty TP53 gene
Cancer type LFS risk (up to age 20) General population risk (up to age 20)
Overall cancerFemales: 15 - 27%
Males: 25 - 39%		< 0.4%
Soft tissue cancer
(sarcoma)		Females: 2.5 - 5%
Males: 6 - 12%		0.02%
Bone cancer
(osteosarcoma)		Females: 2.5 - 7.5%
Males: 7.5 - 12.5%		Rare
Brain
(various)		Up to 8%0.04%
Cancer of the adrenal cortex
(adrenocortical carcinoma)		Up to 9%Rare

The risk of other cancers including leukaemia, Wilms tumour, neuroblastoma, and lung, colorectal, stomach and pancreatic cancers may also be increased (risk figures not quantified).

Note: The conditions associated with LFS and their risk estimates may change as more information is available. Published literature may overestimate cancer risks. These figures are based on retrospective analysis which may be biased towards families with an excess of cancer diagnoses.

Lifetime cancer risks for individuals with a faulty TP53 gene
Cancer type LFS risk (up to age 70) General population risk (up to age 70)
Overall cancer (female)> 95%22%
Overall cancer (male)> 90%23%
Breast (female)85%13%
Soft tissue cancer (sarcoma)50%Rare
Bone cancer (osteosarcoma)10%Rare
Brain cancer20 - 30%Rare
Colorectal cancerUp to 24%4%
Gastric cancer1 - 16%~1%
Cancer of the adrenal cortex (adrenocortical carcinoma)IncreasedRare
Others: Lung, prostate, renal, melanoma and pancreatic cancersIncreased-
Risk of second primary cancer50% by 10 years8%

Note: The conditions associated with LFS and their risk estimates may change as more information is available. Published literature may overestimate cancer risks. These figures are based on retrospective analysis which may be biased towards families with an excess of cancer diagnoses.

Li-Fraumeni Syndrome - How to prevent?

Li-Fraumeni Syndrome - Preparing for surgery

Li-Fraumeni Syndrome - Post-surgery care

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