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:: (A) Peutz-Jeghers syndrome, (B) Hereditary pancreatitis, (C) Familial atypical multiple-mole melanoma syndrome, (D) Hereditary breast and ovarian cancer
Answer is A. 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