Familial clustering of pancreatic cancer has been repoed, and a growing number of inherited genetic defects are recognized to increase pancreatic cancer risk. The disorder with maximum risk of pancreatic cancer by the age of 70 is:
Inherited Predisposing to Pancreatic Cancer Disorder Gene Increased Risk of pancreatic Cancer (Fold) Risk of Pancreatic Cancer by Age 70(%) Peutz-Jeghers syndrome STK11 130 30-60 Hereditary pancreatitis PRSS1, SPINK1 50-80 25-40 Familial atypical multiple-mole melanoma syndrome(FAMMM) CDKN2A 200-35 10-17 Strong family history (3 or more relatives with pancreatic cancer) Unknown 14-32 8-16 HBOC BRCA1, BRCA2, PALP2, BRCA2 4-10 5 HNPCC MLH1, MSH2 (2p21) 8-10 4
Peutz-Jeghers syndrome
Hereditary pancreatitis
Familial atypical multiple-mole melanoma syndrome
Hereditary breast and ovarian cancer
Miscellaneous