MSI-H occurs in 1–3% of pancreatic cancers, which is commonly associated with mutations in the MSH2 and MLH1 MMR genes, and can be detected by immunohistochemistry (MSH1, PMS2, MLH1, and MSH6 expression) or sequencing (single gene mutations and MMR mutational signature) (Waddell et al., 2015; Connor et al., 2017). Here, MLH1 is linked to familial pancreatic carcinoma.