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Multigene germline panel tests (MGPTs) have revealed that 7%\u201318% of patients with pancreatic cancer (PC) harbor pathogenic germline variants (PGVs), almost equal to the levels of breast, ovarian, endometrial, and colorectal cancers, with a higher incidence in FPC (14%\u201326%). The majority of PGVs seen in PC patients are clinically actionable and associated with homologous recombination (HR) pathways (6%\u201310%, particularly <jats:italic>BRCA1\/2<\/jats:italic> in 5%\u20136%), and the clinical guidelines recommend or propose genetic testing for all PC patients. Consensus guidelines have been established for most of the hereditary syndromes associated with PC risks, and surveillances of the pancreas and other at\u2010risk organs are recommended for PGV carriers. Hereditary breast and ovarian cancer (HBOC) is the commonest hereditary cancer syndrome that has moderately increasing life\u2010time risks of PC (3%\u20137% in Western countries); however, recent Japanese research demonstrated a higher risk level (<jats:italic>BRCA1<\/jats:italic>: 16%, <jats:italic>BRCA2<\/jats:italic>: 14%). Moreover, recent evidence has suggested a risk linkage between PC and ovarian cancer in HBOC pedigrees. High scores of homologous recombination deficiency suggest biallelic dysfunction of <jats:italic>BRCA<\/jats:italic> or other HR\u2010related genes, and the likely effectiveness of platinum agents and PARP inhibitors against PCs. Remote counseling and testing are possible option in the future genetic medicine. As PC ranks in the second commonest target of precision cancer medicine in Japan, we must treat the patients and manage their at\u2010risk relatives efficiently.<\/jats:p>","DOI":"10.1002\/jhbp.12112","type":"journal-article","created":{"date-parts":[[2025,1,16]],"date-time":"2025-01-16T02:58:52Z","timestamp":1736996332000},"page":"212-227","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Genetic medicine of familial and hereditary pancreatic cancer: Recent update in the era of precision cancer medicine"],"prefix":"10.1002","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7050-2351","authenticated-orcid":false,"given":"Hiroyuki","family":"Matsubayashi","sequence":"first","affiliation":[{"name":"Division of Genetic Medicine Promotion Shizuoka Cancer Center  Shizuoka Japan"},{"name":"Division of Endoscopy Shizuoka Cancer Center  Shizuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshimi","family":"Kiyozumi","sequence":"additional","affiliation":[{"name":"Division of Genetic Medicine Promotion Shizuoka Cancer Center  Shizuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Ono","sequence":"additional","affiliation":[{"name":"Division of Endoscopy Shizuoka Cancer Center  Shizuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,1,15]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00535-011-0457-z"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnci\/djac167"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10147-023-02317-x"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/gutjnl-2019-319352"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s10038-023-01153-1"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.12490"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2017.85"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0299276"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2024.114226"},{"key":"e_1_2_11_11_1","first-page":"1211","article-title":"Pancreatic cancer screening is effective in individuals at risk with predisposing germline gene variants, but not in gene variant\u2010negative familial pancreatic cancer families. 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