What is the role of Li-Fraumeni syndrome in the development of pediatric breast disorders?

Updated: Apr 01, 2019
  • Author: Harsh Grewal, MD, FACS, FAAP; Chief Editor: Robert K Minkes, MD, PhD  more...
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Answer

Other groups are known to have an increased susceptibility to breast cancer. Families with Li-Fraumeni syndrome have p53 mutations, which are associated with an increased risk for sarcomas, breast cancer, lung cancer, laryngeal cancer, leukemia, and adrenal cortical carcinoma. [35] The pattern of transmission is autosomal dominant. Breast cancer develops in 77% of women with Li-Fraumeni syndrome between age 22 and 45 years, with 25% developing bilateral disease. Rarely, tumors may develop in the teenaged patient.


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