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. About 2–3% of osteosarcomas, 9% of rhabdomyosarcomas, and 7–20% of patients with multiple primary tumours have p53 mutations. Although most cases of this syndrome have early onset of cancer, cases have also been reported later in life. Diagnosis Li–Fraumeni syndrome is diagnosed if these three criteria are met: The patient has been diagnosed with a sarcoma at a young age (below 45). A first-degree relative has been diagnosed with any cancer at a young age (below 45). Another first- or a second-degree relative has been diagnosed with any cancer at a young age (below 45) or with a sarcoma at any age. Other criteria have also been proposed: A proband with any childhood cancer or sarcoma, brain tumor or adrenal cortical carcinoma diagnosed before age 45 A first- or second-degree relative with a typical LFS malignancy (sarcoma, leukaemia, or cancers of the breast, brain or adrenal cortex) regardless of age at diagnosis A first- or second-degree relative with any cancer diagnosed before age 60 A third criterion has been proposed: Two first- or second-degree relatives with LFS-related malignancies at any age. Management Genetic counseling and genetic testing are used to confirm that somebody has this gene mutation. Once such a person is identified, early and regular screenings for cancer are recommended for them as people with Li–Fraumeni are likely to develop another primary malignancy at a future time (57% within 30 years of diagnosis). Chompret criteria A 2015 revision of the traditional Chompret criteria for screening has