Sung H, Ferlay J, Siegel RL, Laversanne M, Soerjomataram I, Jemal A, et al. Global Cancer statistics 2020: GLOBOCAN estimates of incidence and mortality worldwide for 36 cancers in 185 countries. CA Cancer J Clin. 2021;71:209–49.

PubMed  Google Scholar 

Midha S, Chawla S, Garg PK. Modifiable and non-modifiable risk factors for pancreatic cancer: a review. Cancer Lett. 2016;381:269–77.

CAS  PubMed  Google Scholar 

Rawla P, Sunkara T, Gaduputi V. Epidemiology of pancreatic Cancer: global trends, etiology and risk factors. World J Oncol. 2019;10:10–27.

PubMed  PubMed Central  Google Scholar 

Llach J, Carballal S, Moreira L. Familial pancreatic Cancer: current perspectives. Cancer Manag Res. 2020;12:743–58.

CAS  PubMed  PubMed Central  Google Scholar 

Chen F, Childs EJ, Mocci E, Bracci P, Gallinger S, Li D, et al. Analysis of heritability and genetic architecture of pancreatic Cancer: a PanC4 study. Cancer Epidemiol Biomark Prev. 2019;28:1238–45.

CAS  Google Scholar 

Fulton AJ, Lamarca A, Nuttall C, McCallum L, Pihlak R, O'Reilly D, et al. Identification of patients with pancreatic adenocarcinoma due to inherited mutation: challenges of daily clinical practice. World J Gastrointest Oncol. 2019;11:102–16.

PubMed  PubMed Central  Google Scholar 

Scully R, Livingston DM. In search of the tumor-suppressor functions of BRCA1 and BRCA2. Nature. 2000;408:429–32.

CAS  PubMed  PubMed Central  Google Scholar 

Bayraktar S, Arun B. BRCA mutation genetic testing implications in the United States. Breast. 2017;31:224–32.

PubMed  Google Scholar 

Greer JB, Whitcomb DC. Role of BRCA1 and BRCA2 mutations in pancreatic cancer. Gut. 2007;56:601–5.

CAS  PubMed  Google Scholar 

Iqbal J, Ragone A, Lubinski J, Lynch HT, Moller P, Ghadirian P, et al. Hereditary breast Cancer study group. The incidence of pancreatic cancer in BRCA1 and BRCA2 mutation carriers. Br J Cancer. 2012;107:2005–9.

CAS  PubMed  PubMed Central  Google Scholar 

Mocci E, Milne RL, Méndez-Villamil EY, Hopper JL, John EM, Andrulis IL, et al. Risk of pancreatic cancer in breast cancer families from the breast cancer family registry. Cancer Epidemiol Biomark Prev. 2013;22:803–11.

CAS  Google Scholar 

Kim SH, Hwang HK, Lee WJ, Kang CM. Biological behavior of resected BRCA-mutated pancreatic cancer: comparison with sporadic pancreatic cancer and other BRCA-related cancers. Pancreatology. 2021;S1424-3903(21):00057.

Google Scholar 

Lubinski J, Phelan CM, Ghadirian P, Lynch HT, Garber J, Weber B, et al. Cancer variation associated with the position of the mutation in the BRCA2 gene. Familial Cancer. 2004;3:1–10.

CAS  PubMed  Google Scholar 

Al-Sukhni W, Rothenmund H, Borgida AE, Zogopoulos G, O'Shea AM, Pollett A, et al. Germline BRCA1 mutations predispose to pancreatic adenocarcinoma. Hum Genet. 2008;124:271–8.

CAS  PubMed  Google Scholar 

Axilbund JE, Argani P, Kamiyama M, Palmisano E, Raben M, Borges M, et al. Absence of germline BRCA1 mutations in familial pancreatic cancer patients. Cancer Biol Ther. 2009;8:131–5.

CAS  PubMed  Google Scholar 

Lawniczak M, Gawin A, Białek A, Lubiński J, Starzyńska T. Is there any relationship between BRCA1 gene mutation and pancreatic cancer development? Pol Arch Med Wewn. 2008;118:645–9.

CAS  PubMed  Google Scholar 

Golan T, Kindler HL, Park JO, Reni M, Macarulla T, Hammel P, et al. Geographic and ethnic heterogeneity of germline BRCA1 or BRCA2 mutation prevalence among patients with metastatic pancreatic Cancer screened for entry into the POLO trial. J Clin Oncol. 2020;38:1442–54.

CAS  PubMed  Google Scholar 

Pihlak R, Valle JW, McNamara MG. Germline mutations in pancreatic cancer and potential new therapeutic options. Oncotarget. 2017;8:73240–57.

PubMed  PubMed Central  Google Scholar 

Ducy M, Sesma-Sanz L, Guitton-Sert L, Lashgari A, Gao Y, Brahiti N, et al. The tumor suppressor PALB2: inside out. Trends Biochem Sci. 2019;44:226–40.

CAS  PubMed  Google Scholar 

Hofstatter EW, Domchek SM, Miron A, Garber J, Wang M, Componeschi K, et al. PALB2 mutations in familial breast and pancreatic cancer. Familial Cancer. 2011;10:225–31.

CAS  PubMed  Google Scholar 

Potrony M, Badenas C, Aguilera P, Puig-Butille JA, Carrera C, Malvehy J, et al. Update in genetic susceptibility in melanoma. Ann Transl Med. 2015;3:210.

PubMed  PubMed Central  Google Scholar 

Lynch HT, Shaw TG. Familial atypical multiple mole melanoma (FAMMM) syndrome: history, genetics, and heterogeneity. Familial Cancer. 2016;15:487–91.

PubMed  Google Scholar 

Lynch HT, Fusaro RM, Lynch JF, Brand R. Pancreatic cancer and the FAMMM syndrome. Familial Cancer. 2008;7:103–12.

CAS  PubMed  Google Scholar 

Goldstein AM, Chan M, Harland M, Gillanders EM, Hayward NK, Avril MF, et al. Melanoma genetics consortium (GenoMEL). High-risk melanoma susceptibility genes and pancreatic cancer, neural system tumors, and uveal melanoma across GenoMEL. Cancer Res. 2006;66:9818–28.

CAS  PubMed  Google Scholar 

Vasen HF, Gruis NA, Frants RR, van Der Velden PA, Hille ET, Bergman W. Risk of developing pancreatic cancer in families with familial atypical multiple mole melanoma associated with a specific 19 deletion of p16 (p16-Leiden). Int J Cancer. 2000;87:809–11.

CAS  PubMed  Google Scholar 

Ibrahim I, Sibinga Mulder BG, Bonsing B, Morreau H, Farina Sarasqueta A, Inderson A, et al. de Vos Tot Nederveen Cappel W, Wasser M, Vasen HFA. Risk of multiple pancreatic cancers in CDKN2A-p16-Leiden mutation carriers. Eur J Hum Genet. 2018;26:1227–9.

CAS  PubMed  PubMed Central  Google Scholar 

Wagner A, Aretz S, Auranen A, Bruno MJ, Cavestro GM, Crosbie EJ, et al. The Management of Peutz-Jeghers Syndrome: the European hereditary tumour group (EHTG) guideline. J Clin Med. 2021;10:473.

PubMed  PubMed Central  Google Scholar 

Beggs AD, Latchford AR, Vasen HF, Moslein G, Alonso A, Aretz S, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010;59:975–86.

CAS  PubMed  Google Scholar 

Resta N, Pierannunzio D, Lenato GM, Stella A, Capocaccia R, Bagnulo R, et al. Cancer risk associated with STK11/LKB1 germline mutations in Peutz-Jeghers syndrome patients: results of an Italian multicenter study. Dig Liver Dis. 2013;45:606–11.

CAS  PubMed  Google Scholar 

Choi HS, Park YJ, Youk EG, Yoon KA, Ku JL, Kim NK, et al. Clinical characteristics of Peutz-Jeghers syndrome in Korean polyposis patients. Int J Color Dis. 2000;15:35–8.

CAS  Google Scholar 

Iwamuro M, Toyokawa T, Moritou Y, Matsueda K, Hori S, Yoshioka M, et al. Peutz-Jeghers syndrome and cancer:a retrospective study in 14 Japanese patients with Peutz-Jeghers syndrome. Nihon Shokakibyo Gakkai Zasshi. 2019;116:1015–21.

PubMed  Google Scholar 

Maratt JK, Stoffel E. Identification of Lynch syndrome. Gastrointest Endosc Clin N Am. 2022;32:45–58.

PubMed  Google Scholar 

Kuiper RP, Vissers LE, Venkatachalam R, Bodmer D, Hoenselaar E, Goossens M, et al. Recurrence and variability of germline EPCAM deletions in Lynch syndrome. Hum Mutat. 2011;32:407–14.

CAS  PubMed  Google Scholar 

Dymerska D, Gołębiewska K, Kuświk M, Rudnicka H, Scott RJ, Billings R, et al. New EPCAM founder deletion in polish population. Clin Genet. 2017;92:649–53.

CAS  PubMed  Google Scholar 

Dymerska D, Kurzawski G, Suchy J, Roomere H, Toome K, Metspalu A, et al. Lynch syndrome mutations shared by the Baltic States and Poland. Clin Genet. 2014;86:190–3.

CAS  PubMed  Google Scholar 

Sinicrope FA. Lynch syndrome-associated colorectal Cancer. N Engl J Med. 2018;379:764–73.

CAS  PubMed  Google Scholar 

Bujanda L, Herreros-Villanueva M. Pancreatic Cancer in Lynch syndrome patients. J Cancer. 2017;8:3667–74.

PubMed  PubMed Central  Google Scholar 

Dinarvand P, Davaro EP, Doan JV, Ising ME, Evans NR, Phillips NJ, et al. Familial adenomatous polyposis syndrome: an update and review of Extraintestinal manifestations. Arch Pathol Lab Med. 2019;143:1382–98.

CAS  PubMed  Google Scholar 

Giardiello FM, Offerhaus GJ, Lee DH, Krush AJ, Tersmette AC, Booker SV, et al. Increased risk of thyroid and pancreatic carcinoma in familial adenomatous polyposis. Gut. 1993;34:1394–6.

CAS  PubMed  PubMed Central  Google Scholar 

Lepistö A, Kiviluoto T, Halttunen J, Järvinen HJ. Surveillance and treatment of duodenal adenomatosis in familial adenomatous polyposis. Endoscopy. 2009;41:504–9.

PubMed  Google Scholar 

Bülow S, Christensen IJ, Højen H, Björk J, Elmberg M, Järvinen H, et al. Duodenal surveillance improves the prognosis after duodenal cancer in familial adenomatous polyposis. Color Dis. 2012;14:947–52.