The Li-Fraumeni syndrome section was comprehensively reviewed and extensively revised.
Revised text to state that an updated set of operational
criteria for the diagnosis of Cowden syndrome based on a
systematic literature review has been suggested and is
currently utilized in the National Comprehensive Cancer Network (NCCN) guidelines.
Added text to state that despite discordant findings regarding
RRSO and breast cancer risk in the existing literature,
aggregate data suggest that there is a benefit, although the
magnitude of this benefit may not be fully understood.
Further prospective studies are needed to confirm these
findings.
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