Review
Copyright ©2014 Baishideng Publishing Group Inc.
World J Gastroenterol. Sep 14, 2014; 20(34): 12118-12131
Published online Sep 14, 2014. doi: 10.3748/wjg.v20.i34.12118
Table 1 Non-genetic factors associated with an increased risk to develop pancreatic cancer
Risk factorsEstimated overall riskRef.
Smoking1.75[37,38]
Overweight1.12 per increased 5 kg/m2[39-41]
Alchohol abuse1.2[42,43]
Type 1 diabetes2.0[44]
New onset type 2 diabetes2.0[45]
Chronic pancreatitis14.0[46]
Exposure to nickel1.9[47]
Previous gastric ulcer1.8[48]
Table 2 Most important genetic syndromes associated with an increased risk of pancreatic cancer
SyndromeGeneRelative riskRisk at age of 70
Familial pancreatic cancerUnknown
1 or more first degree relative(s)94%
1 first degree relative4.52%
2 first degree relatives6.43%
3 or more first degree relatives3216%
Peutz Jeghers syndromeLKB1/STK1113230%-60%
Hereditary pancreatitisPRSS150-7040%
Familial atypical multiple mole melanomaCDKN2A/p1634-3917%
Breast and ovarian cancer syndromeBRCA1/BRCA22.3-101%-5%
Cystic fibrosisCFTR5.3< 5%
Hereditary non-polyposis colon cancerMSH2, MLH1, MSH6, PMS, PMS24.7< 5%
Familial adenomatous polyposisAPC4.52%
Table 3 Characteristics related to epithelial subtype in intraductal papillary mucinous neoplasia
Epithelial subtype
GastricIntestinalPancreatobiliaryOncocytic
LocationBranch duct > main ductMain duct > branch ductBranch duct > main ductBranch duct > main duct
DysplasiaLGD/IGDIGD/HGDHGDHGD
Present with invasive carcinoma15%30%-60%60%-75%25%
Type of invasive carcinomaConventional (tubular)Colloid or conventional (tubular)Conventional (tubular)Oncocytic or conventional (tubular)
Table 4 Incidence of cancer during follow-up of branch-duct intraductal papillary mucinous neoplasms of the pancreas
Ref.YearPatients (n)Incidence of cancer on IPMN
Lafemina et al[97]201317011.0%
Sahora et al[98]20132404.0%1
Khannoussi et al[99]2012533.7%
Arlix et al[100]2012490
Maguchi et al[101]20113492.6%2
Salvia et al[102]20091210
Tanno et al[103]2008821.2%
Table 5 Summary of diagnostic yield of familial pancreatic cancer screening
Ref.YearSyndrome(s)Patients (n)Yield
Brentnall et al[106]1999FPC1450%
Kimmey et al[107]2002FPC4626%
Poley et al[108]2009FPC, PJS, BRCA, p16, p53, HP4423%
Langer et al[109]2009FPC, BRCA761.3%
Verna et al[110]2010FPC, BRCA, p165112%
Ludwig et al[111]2011FPC, BRCA1098.3%
Vasen et al[112]2011P167918%
Al-Sukhni et al[113]2011FPC, BRCA, PJS, p16, HP2627.3%
Schneider et al[114]2011FPC, BRCA, PALB27215%
Canto et al[115]2012FPC, BRCA, PJS21643%
Table 6 Surgical procedure performed in patients at ”hereditary risk” for pancreatic cancer (n)
Ref.YearResectedPanIN1PanIN2PanIN3Pancreas cancerOther bening lesionsOther malignant lesionsBenign IPMNMalignant or high-grade displasia IPMNMalignant lesions or high-grade dysplasia1
Brentnall et al[106]20067-22--2142.8%
Poley et al[108]20093---3---100%
Verna et al[110]20105---1--4-20%
Ludwig et al[111]201161111--2-16.7%
Vasen et al[112]20117---7----100%
Al-Sukhni et al[113]20114---1-12-50%
Schneider et al[114]2011911113-2-22.2%
Canto et al[115]20125--1---3140%
Total46245143115247.8%