title: The incidence of pancreatic cancer in BRCA1 and BRCA2 mutation carriers.
abstract: BACKGROUND
  Germline mutations in BRCA1 and BRCA2 predispose to pancreatic cancer. We estimated the incidence of pancreatic cancer in a cohort of female carriers of BRCA1 and BRCA2 mutation. We also estimated survival rates in pancreatic cancer cases from families with a BRCA mutation.
  
  
  METHODS
  We followed 5149 women with a mutation for new cases of pancreatic cancer. The standardised incidence ratios (SIR) for pancreatic cancer were calculated based on age group and country of residence. We also reviewed the pedigrees of 8140 pedigrees with a BRCA1 or a BRCA2 mutation for those with a case of pancreatic cancer. We recorded the year of diagnosis and the year of death for 351 identified cases.
  
  
  RESULTS
  Eight incident pancreatic cancer cases were identified among all mutation carriers. The SIR for BRCA1 carriers was 2.55 (95% CI=1.03-5.31, P=0.04) and for BRCA2 carriers was 2.13 (95% CI=0.36-7.03, P=0.3). The 5-year survival rate was 5% for cases from a BRCA1 family and 4% for cases from a BRCA2 family.
  
  
  CONCLUSIONS
  The risk of pancreatic cancer is approximately doubled in female BRCA carriers. The poor survival in familial pancreatic cancer underscores the need for novel anti-tumoural strategies.
authors.affiliation: Women's College Research Institute, Familial Breast Cancer Research, 790 Bay Street, Toronto, Ontario, Canada M5G 1N8.
authors.forename: J
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authors.lastname: Iqbal
authors.forename: A
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authors.lastname: Ragone
authors.forename: J
authors.initials: J
authors.lastname: Lubinski
authors.forename: H T
authors.initials: HT
authors.lastname: Lynch
authors.forename: P
authors.initials: P
authors.lastname: Moller
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authors.initials: P
authors.lastname: Ghadirian
authors.forename: W D
authors.initials: WD
authors.lastname: Foulkes
authors.forename: S
authors.initials: S
authors.lastname: Armel
authors.forename: A
authors.initials: A
authors.lastname: Eisen
authors.forename: S L
authors.initials: SL
authors.lastname: Neuhausen
authors.forename: L
authors.initials: L
authors.lastname: Senter
authors.forename: C F
authors.initials: CF
authors.lastname: Singer
authors.forename: P
authors.initials: P
authors.lastname: Ainsworth
authors.forename: C
authors.initials: C
authors.lastname: Kim-Sing
authors.forename: N
authors.initials: N
authors.lastname: Tung
authors.forename: E
authors.initials: E
authors.lastname: Friedman
authors.forename: M
authors.initials: M
authors.lastname: Llacuachaqui
authors.forename: S
authors.initials: S
authors.lastname: Ping
authors.forename: S A
authors.initials: SA
authors.lastname: Narod
mesh_terms: D000328:Adult; D000368:Aged; D019313:BRCA1 Protein / Q000235:genetics*; D024682:BRCA2 Protein / Q000235:genetics*; D015331:Cohort Studies; D005260:Female; D019398:Genes, BRCA1; D024522:Genes, BRCA2; D018095:Germ-Line Mutation; D006579:Heterozygote; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000453:epidemiology* / Q000235:genetics* / Q000401:mortality
chemical_list: D019313:BRCA1 Protein; C492913:BRCA1 protein, human; D024682:BRCA2 Protein; C551750:BRCA2 protein, human