There are limited recommendations on this topic.¹ The following summarizes the literature, however, a clinician’s judgment will also be based on the patient’s age, co-morbidities and individual circumstances.

Management after risk-reducing mastectomy

Prophylactic mastectomy reduces the risk of breast cancer in BRCA carriers by about 90%,²<img although a recent prospective study demonstrated a 0.8% annual incidence of breast cancer in these patients.³ The risk reduction is about 95% for women who have also undergone bilateral salpingo-oophorectomy prior to menopause.² The National Comprehensive Cancer Network (NCCN) advocates annual physical examinations following bilateral mastectomy, but does not make recommendations regarding breast imaging.⁴ Zakhireh et al. concluded that the residual risk of a non-palpable breast cancer does not justify surveillance by mammography or MRI.⁵ Breast cancer risk-lowering drugs such as tamoxifen are unlikely to provide additional benefit.² 

Management after risk-reducing salpingo-oophorectomy

Historical data suggest that women at high risk for ovarian cancer who undergo prophylactic bilateral salpingo-oophorectomy face about a 2% to 11% risk for primary peritoneal carcinoma (PPC).^6,7 However, more recent data suggest that this risk may be lower when a rigorous surgical-pathological protocol is followed to detect and treat occult cancer at the time of surgery.⁸ NCCN guidelines9 do not include recommendations for monitoring this risk, and the American Congress of Obstetricians and Gynecologists considers surveillance to be investigational.¹⁰ Chapman et al. recently proposed guidelines for following these patients, including pelvic exams, consideration of CA-125 and counseling regarding symptoms of PPC, as well management of bone health and menopausal symptoms.¹¹

Additional Cancer Risks

In addition to breast and ovarian cancer, women with BRCA mutations are at elevated risk for melanoma and pancreatic cancer. Guidelines suggest considering annual full body skin examinations for melanoma and investigational protocols for pancreatic cancer screening.⁹

Comment

Many patients experience great relief once a decision has been made and surgery completed. However, it is important for both patient and healthcare professional to understand that prophylactic surgery leads to significant, but not total reduction in risk. As more experience is gained with Hereditary Breast and Ovarian Cancer Syndrome, information about residual risk and appropriate management will become clearer. This summary of the current literature and guidelines provides a basic foundation from which the provider and patient can build a management plan.