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Access : The incidence of pancreatic cancer in BRCA1 and BRCA2 mutation carriers : British Journal of Cancer
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.
Conclusion:
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.
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