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Objective  Conflicting conclusions have been published regarding breast cancer survival of  BRCA1/2  mutation carriers. Here we provide an evidence-based systematic literature review.    Methods  Eligible publications were observational studies assessing the survival of breast cancer patients carrying a  BRCA1/2  mutation compared to non-carriers or the general breast cancer population. We performed meta-analyses and best-evidence syntheses for survival outcomes taking into account study quality assessed by selection bias, misclassification bias and confounding.    Results  Sixty-six relevant studies were identified. Moderate evidence for a worse unadjusted recurrence-free survival for  BRCA1  mutation carriers was found. For  BRCA1  and  BRCA2  there was a tendency towards a worse breast cancer-specific and overall survival, however, results were heterogeneous and the evidence was judged to be indecisive. Surprisingly, only 8 studies considered adjuvant treatment as a confounder or effect modifier while only two studies took prophylactic surgery into account. Adjustment for tumour characteristics tended to shift the observed risk estimates towards a relatively more favourable survival.    Conclusions  In contrast to currently held beliefs of some oncologists, current evidence does not support worse breast cancer survival of  BRCA1/2  mutation carriers in the adjuvant setting; differences if any are likely to be small. More well-designed studies are awaited.

Worse Breast Cancer Prognosis of BRCA1/BRCA2 Mutation Carriers: What's the Evidence? A Systematic Review with Meta-Analysis