The Role of Risk-Reducing Surgery in Hereditary Breast and Ovarian Cancer

HEREDITARY BREAST AND OVARIAN CANCER IS A SYNDROME THAT INvolves an increased predisposition to breast cancer, ovarian cancer, or both and an autosomal dominant pattern of transmission. The numbers of breast-cancer diagnoses, the ages of patients at diagnosis, and the occurrence of ovarian cancer in addition to breast cancer vary among families with hereditary breast and ovarian cancer syndrome. The likelihood of detecting an underlying disease-causing mutation is highest in the most severely affected families, especially those with ovarian cancer. Disease-causing mutations in BRCA1 and BRCA2, the genes most often associated with hereditary breast and ovarian cancer syndrome, are identified in only a minority of families with suspected hereditary breast and ovarian cancer syndrome. Risk-reducing mastectomy and risk-reducing salpingo-oophorectomy are options for the primary prevention of breast and ovarian cancers, and they have been shown in multiple studies to have efficacy. However, these procedures, which have profound effects on a woman's body, are associated with complex and emotionally charged decision making. In this review, we address issues related to the care of women in families with hereditary breast and ovarian cancer syndrome who have not had cancer. We discuss risk assessment for breast and ovarian cancers according to the woman's age, the efficacy of risk-reducing surgery, the complications and psychosocial effects of these procedures, alternative strategies for risk management, and the best ways to facilitate individual decision making.