These hereditary cancer syndromes include pathogenic variant carriers of STK11, PRSS1, BRCA2, MMR (MLH1, MSH2, MSH6, PMS2), PALB2, and ATM. As the CDKN2A/p16 variant carriers (FAMMM) has rarely been reported and the guidelines for FAMMM have not been established in Japan, the Japanese Clinical Guidelines for Pancreatic Cancer (2022) [16] did not state exactly on the method of surveillance. Here, MSH6 is linked to pancreatic neoplasm.