Table 1.
Syndrome | Gene | RR | Lifetime risk |
Peutz-Jeghers syndrome | STK11/LKB1 | 132 | 36% by age 65 yr |
Hereditary pancreatitis | PRSS1 | 53 | Male: 11% and 49% by age 50 and 75 yr |
Female: 8% and 55% by age 50 and 75 yr | |||
FAMMM | p16 | 13-22 | 16% lifetime risk |
Familial breast and ovarian | BRCA1/2 | 3-10 | 5% lifetime risk |
HNPCC | MLH1, MSH6, MSH2, PMS2 | 1.5-9 | 8.6% lifetime risk |
Familial pancreatic cancer | |||
2 FDR | Unknown | 6.4 | 8%-12% lifetime risk |
3 FDR | Unknown | 32 | 40% lifetime risk |
FDR: First degree relative; HNPCC: Hereditary non-polyposis colorectal cancer; PRSS1: Cationic trypsinogen gene; FAMMM: Familial atypical multiple mole melanoma syndrome.