Chompret criteria
A 2015 revision of the traditional Chompret criteria for screening has been proposed - a proband who has:
A tumor belonging to LFS tumor spectrum (e.g., premenopausal breast cancer, soft tissue sarcoma, osteosarcoma, CNS tumor, adrenocortical carcinoma) before age 46 years, and at least one first- or second-degree relative with LFS tumor (except breast cancer if the proband has breast cancer) before age 56 years or with multiple tumors at any age
Multiple tumors (except multiple breast tumors), two of which belong to LFS spectrum with the initial cancer occurring before the age of 46 years
An adrenocortical carcinoma, choroid plexus tumor, or rhabdomyosarcoma of embryonal anaplastic subtype, at any age of onset, irrespective of family history
Breast cancer before age 31

Recommendations
Recommendations for individuals from families affected by the syndrome include:
Avoidance of radiation therapy to reduce risk of secondary radiation-induced malignancies
Children and adults undergo comprehensive annual physical examination
Women undergo age-specific breast cancer monitoring beginning at age 25 years
All patients consult a physician promptly for evaluation of lingering symptoms and illnesses

Suggestions
Adults should undergo routine screening for colorectal cancer beginning no later than age 25 years. Individuals should undergo organ-targeted surveillance based on the pattern of cancer observed in their families. Prophylactic mastectomy to reduce the risk of breast cancer is an option. Epidemiology
Li–Fraumeni syndrome (LFS) is rare; as of 2011, cases had been reported in more than 500 families. The syndrome was discovered using an epidemiological approach.
Clinical
The classical LFS malignancies - sarcoma, cancers of the breast, brain, and adrenal glands - comprise about 80% of all cancers that occur in this syndrome. The risk of developing any invasive cancer (excluding skin cancer) is about 50% by age 30 (1% in the general population) and is 90% by age 70. Early-onset breast cancer accounts for 25% of all the cancers in this syndrome. This is followed by soft-tissue sarcomas (20%), bone sarcoma (15%), and brain tumors - especially glioblastomas - (13%). Other tumours seen in this syndrome include leukemia, lymphoma, and adrenocortical carcinoma. About 90% of females with LFS develop breast cancer by age 60 years; the majority of these occur before age 45 years. Females with this syndrome have almost a 100% lifetime risk of developing cancer. This compares with 73% for affected males. The difference may be due to much smaller breast tissue in males and increased estrogen levels in females. The risks of sarcoma, female breast cancer, and haematopoietic malignancies in mutation carriers are more than 100 times greater than those seen in the general population. Other tumours reported in this syndrome, but not yet proved to be linked with it, include melanoma, Wilms and other kidney tumors, hepatocellular carcinoma, gonadal germ cell, pancreatic, gastric, choroid plexus, colorectal, and prostate cancers. Around 80% of children with adrenocortical carcinoma and 2–10% of childhood brain tumors have p53 mutations.