Table 5.
Prevalence/incidence of IPMNs and pancreatic cancer in hereditary genetic predisposition syndromes.
| Gene Mutation | IPMNs | PC | |
|---|---|---|---|
| MAS | GNAS | 16–46% [81,82] | 1 case [83] |
| Lynch | MLH1, MSH2, MSH6 | 3 cases [84,85,86] | 0.7–3.7% [87,88] |
| PJS | STK11/LKB1 | 100% [89] | 11–36% [90] |
| FAP | APC, MUTYH | 3 cases [91,92,93] | 3% (4/127 including 2 endocrine carcinomas, 1 acinar cell carcinoma, 1 pancreatoblastoma) [94] |
| CNC | PRKAR1A | 0.8% [95] | 1.7% (6/354 with only 1 PDAC) [95] |
MAS—McCune Albright syndrome; PJS—Peutz Jeghers syndrome; FAP—familial adenomatosis polyposis; CNC—Carney complex syndrome; IPMNs—intraductal papillary mucinous neoplasms; PC—pancreatic cancer; PDAC—pancreatic ductal adenocarcinoma.