From: Surgical and molecular pathology of pancreatic neoplasms
Gene (syndrome) | RR (Cumulative lifetime risk (%) by age 70) |
---|---|
STK11/LKB1 (Peutz-Jeghers syndrome) | 132 (36) |
PRSS1/SPINK1 (hereditary pancreatitis) | 50–80 (40) |
CDKN2A (FAMMM) | 13–47 (17) |
BRCA1/BRCA2 (HBOC) | 3.5–10 (3–8) |
MLH1, MSH2, MSH6, PMS2 (Lynch syndrome) | 8.6 (<5) |
CFTR (cystic fibrosis) | 5 (<5) |
FDR with PC | 2–3 (2) |
FDRs with PC | 6 (8–12) |
Possible role in FPC: ATM, TET2, DNMT3A, POLN, POLQ, ASXL1, PALB2, FANCG, BUB1B, ESCO2, FANCC, FANCM, MSH4, RAD54L | Unknown |