Genetic predisposition to pancreatic cancer

Table 1 Syndromes and genes associated with hereditary predisposition to pancreatic adenocarcinoma, relative and lifetime risk

Settings of hereditary PC	RR of PC (-fold)	Cumulative lifetime risk by age 70 (%)	Genes identified
FPC syndrome			PALLD, CDKN2A, BRCA2, PALB2, ATM,….?
FDR with PC	2-3	2
FDRs with PC	6	8-12
or more FDRs with PC	14-32	40
Hereditary cancer syndromes
PJS	132	36	STK11/LKB1
MPCS/FAMMM	13-47	17	CDKN2A
HBOC	3.5-10	3-8	BRCA1, BRCA2
LS	8.6	< 5	MLH1, MSH2, MSH6
FAP	2-3	< 5	APC
Syndromes of chronic inflammation
HP	50-80	40	PRSS1, SPINK1
CF	5	< 5	CFTR

HP: Hereditary pancreatitis; FAP: Familial adenomatous polyposis; PC: Pancreatic adenocarcinoma; FDR: First-degree relative; PJS: Peutz-Jeghers syndrome; MPCS: Melanoma pancreatic-cancer syndrome; FAMMM: Familial atypical multiple mole melanoma; HBOC: Hereditary breast-ovarian cancer; LS: Lynch syndrome; FPC: Familial PC; CF: Cystic fibrosis; HP: Hereditary pancreatitis.