What is the morbidity and mortality associated with Li-Fraumeni syndrome?

Updated: Nov 13, 2018
  • Author: ; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Answer

The cancers that occur most commonly in members of Li-Fraumeni syndrome kindreds are breast cancer, brain tumors, acute leukemia, soft-tissue sarcomas, osteosarcoma, and adrenal cortical carcinoma. Individuals with Li-Fraumeni syndrome have a lifetime cancer risk that approaches 100% by age 70 years. [20] More than half of all tumors occur before age 30 years. [21] Patients with Li-Fraumeni syndrome can be successfully treated for the initial cancer, however, radiation therapy is avoided, when possible, due to several case reports and preclinical evidence demonstrating an increased risk in radiation-induced cancers in these patients. [22, 23]  Furthermore, Li-Fraumeni patients are at significant risk for the development of a second primary malignancy [24] .  


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