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. Author manuscript; available in PMC: 2021 Jul 15.
Published in final edited form as: Arch Pathol Lab Med. 2009 Mar;133(3):365–374. doi: 10.5858/133.3.365

Table 2.

Pancreatic Cancer-Associated Genetic Syndromes

Genetic Syndrome Gene(s) Pancreatic
cancer risk (fold)
Histopathology
Hereditary breast and ovarian cancer
syndrome
BRCA2
BRCA1
3.5-10
2
Ductal adenocarcinoma
Peutz-Jegher syndrome STK11/LKB1 132 Intraductal papillary mucinous neoplasm
Hereditary pancreatitis PRSS1
SPINK1
53 Ductal adenocarcinoma
Hereditary non-polyposis colorectal cancer
syndrome
Mismatch repair
genes
Increased Medullary carcinoma
Familial atypical multiple mole melanoma CDKN2A 13-22 Ductal adenocarcinoma
Familial adenomatous polyposis APC Up to 4 Ductal adenocarcinoma
Intraductal papillary mucinous neoplasm
Pancreatoblastoma
Familial Pancreatic Cancer Unknown 9-32