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Multigene panel testing for pancreatic cancer detected the germline variants <jats:italic>BRCA1\/2<\/jats:italic>, <jats:italic>PALB2<\/jats:italic>, <jats:italic>ATM<\/jats:italic>, <jats:italic>TP53<\/jats:italic>, <jats:italic>MLH1<\/jats:italic>, <jats:italic>STK11\/LKB1<\/jats:italic>, <jats:italic>APC<\/jats:italic>, <jats:italic>CDKN2A<\/jats:italic>, and <jats:italic>SPINK1\/PRSS1<\/jats:italic> as high-risk genes. A latest genome-wide association study revealed the common, but low-risk germline variants in pancreatic cancer patients. Active pancreatic surveillance using magnetic resonance imaging and endoscopic ultrasound is recommended for high-risk individuals who have a family history of pancreatic cancer or harbor these germline pathogenic variants to improve the detection rate and prognosis of pancreatic cancer. Since poly-ADP-ribose polymerase (PARP) inhibitor has been shown to be effective in improving the prognosis of <jats:italic>BRCA<\/jats:italic>-positive pancreatic cancer as well as hereditary breast and ovarian cancer syndrome, PARP inhibitor therapy is currently being applied as precision medicine to pancreatic cancer patients harboring the <jats:italic>BRCA1\/2<\/jats:italic> germline variant. This review highlights the importance of surveillance for germline pathogenic variants in pancreatic cancer and is expected to lead to improvements in the diagnosis and prevention of pancreatic cancer as well as facilitate the development of effective therapeutic strategies and precision medicine.<\/jats:p>","DOI":"10.1007\/s10147-021-02015-6","type":"journal-article","created":{"date-parts":[[2021,9,3]],"date-time":"2021-09-03T18:23:57Z","timestamp":1630693437000},"page":"1784-1792","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Hereditary pancreatic cancer"],"prefix":"10.1007","volume":"26","author":[{"given":"Kodai","family":"Abe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minoru","family":"Kitago","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Kitagawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4124-5488","authenticated-orcid":false,"given":"Akira","family":"Hirasawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,9,2]]},"reference":[{"issue":"5","key":"2015_CR1","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1097\/MPA.0000000000000816","volume":"46","author":"K Yamaguchi","year":"2017","unstructured":"Yamaguchi K, Okusaka T, Shimizu K et al (2017) Clinical practice guidelines for pancreatic cancer 2016 from the Japan Pancreas Society. 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