Women diagnosed with BRCA1/2 mutation positive breast cancer have an increased lifetime risk of contralateral breast cancer and ovarian cancer. They benefit from risk-reducing surgical strategies such as mastectomy and salpingo-oophorectomy. For patients with BRCA mutations and hormone-receptor positive breast cancer, the option of combined bilateral annexectomy and hormonal therapy with Aromatase Inhibitor can be discussed with high-risk patients. For triple negative breast cancer with BRCA mutation, there is some evidence that adding platinum-agents in the neoadjuvant setting improves the pathologic complete response. Lastly, ongoing clinical trials testing the efficacy of poly (ADP-ribose) polymerase inhibitor therapy in patients with BRCA1/2 mutations will be determinant for the future guideline recommendations in determining best treatment options for these patients.

(BELG J MED ONCOL 2018;12(5):239–246)