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 60A 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).
Sialectasis (also termed sialectasia, or siadochiectasis) is cystic dilation of the ducts of salivary glands. It may be caused by salivary duct strictures or stones (sialolithiasis). It can also rarely be congenital. See also
Bronchiectasis


== References ==