Introduction: The optimal clinical management of breast cancer (BC) arising in BRCA1/2 mutations carriers is a difficult issue complicated by the risk of subsequent malignancies and by the potential differences in response to local and systemic therapies.
Aim: Systematically review the difference in outcome after breast conservation therapy (BCT) and uni-or bilateral mastectomy in BRCA1/2 related BC.
Material and methods: We selected 20 studies, for which we evaluated the methodology, the characteristics of the populations, biases, confounding risk factors and outcomes.
Results: All studies are retrospective, entailed by numerous biases. They varied with respect to patients' number, selection, and confounding factors. Hereditary BC patients carried an increased risk of ipsilateral recurrence in 5/17 studies, a worse survival in 4/14, an increased risk of contralateral BC in 14/16.
Conclusion: Except for contralateral risk, the presence of a BRCA mutation does not seem to offer additional prognostic information. Large prospective trials, stratified for risk reduction strategies are warranted.