Which age groups have the highest prevalence of Li-Fraumeni syndrome-related cancer?

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

Although approximately 10% of cancers occur in individuals younger than 45 years in the general population, more than half of the cancers occur in Li-Fraumeni syndrome family members younger than 45 years.

The risk for developing soft-tissue sarcomas is greatest before age 10 years. Brain cancer also appears early in childhood, with a second peak in the fourth to fifth decade of life. Osteosarcoma risk peaks during adolescence. Breast cancer risk for females with Li-Fraumeni syndrome increases significantly at around age 20 years and continues to increase in adulthood. [20]

Bougeard et al updated the clinical presentation of 1,730 French patients suggestive of LFS by identifying 415 mutation carriers in 214 families harboring 133 distinct TP53 alterations. The study found that in children, the LFS tumor spectrum was characterized by osteosarcomas, adrenocortical carcinomas (ACC), CNS tumors, and soft tissue sarcomas (STS). In adults, the tumor distribution was characterized by the predominance of breast carcinomas observed in 79% of the females, and STS observed in 27% of the patients. [27]


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