The 2015 clinical guideline for hereditary gastrointestinal cancer syndromes by the American College of Gastroenterology (ACG) covers Peutz-Jeghers syndrome (PJS), hereditary pancreatitis (HP), familial atypical multiple mole melanoma (FAMMM), familial adenomatous polyposis (FAP), Lynch syndrome (LS) and hereditary breast and ovarian cancer syndrome (HBOC), together with Li-Fraumeni syndrome harboring TP53 mutation or ataxia-telangiectasia harboring ATM mutation [6]. This evidence concerns the gene TP53 and Peutz-Jeghers syndrome.