Table 4.

The incidence of IPMNs and pancreatic cancer among patients with significant family history of pancreatic cancer.

	HRI	Total	IPMNs	PC
Canto et al. 2002 [65]	FPC or PJS	38	1 (PJS)	1 (FPC)
Canto et al. 2006 [14]	FPC or PJS	78 (72 FPC + 6 PJS) 149 controls	7 1 cyst in control	2
Poley et al. 2009 [67]	FPC or genetic predisposition syndromes	45 (13 FAMMM + 21 FPC + 3 HP + 2 PJS + 3 BRCA1 + 2 BRCA2 + 1 p53)	7	2
Verna et al. 2010 [68]	3 BRs or 2 FDRs or 2 BRs with 1 FDR or genetic predisposition syndrome	24	3	2
Ludwig et al. 2011 [66]	1 FDR or 2 BRs or BRCA with FH of PC	109	5	1 (FDR)
Al-Sukhni et al. 2012 [69]	2 FPCs or genetic predisposition syndromes or HP or FDR of double primary cancer patient	262 (159 FPC + 7 PJS + 68 BRCA2 + 11 p16 + 5 BRCA1 + 2 HP + 10 double primary)	15 (9 FPC, 4 BRCA2, 1 HP, 1 double primary)	3 (2 FPC and 1 BRCA2)
Sud et al. 2014 [70]	2 FDRs or 3 BRs or HP or PJS or p16 or Lynch with FH of PC	16	1	2
Chang et al. 2017 [71]	Any BR	303	47	7/18 (pathological diagnosis)
Gangi et al. 2018 [72]	2 BRs (including 1 FDR) or PJS or HP or FAMMM or BRCA2 mutations with FH	58 (48 ≥ 1 FDR + 9 BRCA2 + 1 PJS)	1 (2 FDR)	0

HRI—high-risk individuals; IPMNs—intraductal papillary mucinous neoplasm; PC—pancreatic cancer; FPC—familial pancreatic cancer; PJS—Peutz Jeghers syndrome; BR—blood relative; FDR—first-degree relative; FH—family history; HP—hereditary pancreatitis; FAMMM—familial atypical multiple mole melanoma syndrome.