Upper Gastrointestinal Cancer Risk and Surveillance Outcomes in Li-Fraumeni Syndrome.
INTRODUCTION
To assess the upper gastrointestinal (UGI) cancer risk and surveillance outcomes in Li-Fraumeni syndrome (LFS).


METHODS
Analysis of the International Agency for Research on Cancer database and a single-center adult LFS cohort.


RESULTS
UGI cancer was present in 7.2% of families and 3.9% of individuals with a pathogenic/likely pathogenic TP53 mutation in International Agency for Research on Cancer; 29% occurred before age 30. Our institutional cohort had 35 individuals (31% of the LFS cohort) with 48 cumulative upper endoscopies; 3 (8.5%) individuals had concerning UGI findings.


DISCUSSION
UGI cancer is observed in LFS. Upper endoscopy should be part of a comprehensive LFS surveillance program.