👉The lifetime ovarian cancer risk is 39% to 59% for BRCA1 mutation carriers and 11% to 18% for BRCA2 carriers.
👉Other genes associated with increased ovarian cancer risk include BRIP1, RAD51C, RAD51D, MSH2, MLH1, PMS2, MSH6, PALB2, and BARD1.
👉BRCA 1 / 2 associated ovarian cancers are high-grade serous or undifferentiated carcinomas that are typically metastatic at presentation.
👉Most of these tumors arise in one of the fallopian tubes.
👉Median age at ovarian cancer diagnosis for BRCA 1 /2 mutation carriers is 51 years.
👉Annual incidence peaks between ages 50 and 59 for BRCA1 carriers and 60 and 69 for BRCA2 carriers.
👉Risk-reducing bilateral salpingo-oophorectomy (RRSO) reduces ovarian cancer risk by 72% to 96% (79% in a recent meta-analysis) in BRCA1/BRCA2 gene mutation carriers and is also associated with a 60% to 77% reduction in all-cause mortality.
👉Specialized protocols for performing the surgery and processing the specimens have been recommended as clinically occult cancer can be identified in 4% to 5%.
👉Case-control and cohort data suggest that bilateral salpingo-oophorectomy also reduces breast cancer risk by 50%.
👉There is some evidence that risk reduction is greater when the procedure is performed at a younger age.
👉The effect may be larger for BRCA2 carriers than for BRCA1 carriers.
👉Some have argued that the breast cancer risk reduction observed with RRSO is a statistical aberration resulting from unmanaged bias.
👉Others have refuted this.
👉For the time being, it seems prudent to recommend RRSO for the ovarian cancer and mortality benefits without relying too heavily on the procedure for breast cancer risk reduction.
👉Because it is increasingly recognized that hereditary ovarian cancers most commonly arise in the tubes and not the ovary proper, there is increasing interest in performing salpingectomy alone and leaving the ovaries intact.
👉There are currently no data quantifying the risk reduction afforded by this approach, and it is unlikely that breast cancer risk would be reduced.
👉However, there is likely merit to this approach; several studies and registries are currently accruing, so more data will be forthcoming.
👉There is also discussion about whether the uterus should be removed at the time of RRSO.
👉Current guidelines do not mandate that it should, but some advocate it to improve the safety of future tamoxifen use.
👉Of note, one recent study reported five serous or serouslike endometrial cancers among 1,083 BRCA1/2 mutation carriers undergoing RRSO and followed for a median of 5 years.
👉Less than one cancer of this type was expected.
👉The current standard of care is to consider bilateral salpingo-oophorectomy after childbearing is complete or about age 35 to 40.
👉NCCN guidelines recommend consideration of risk-reducing salpingo-oophorectomy for women found to carry pathogenic mutations in BRCA1, BRCA2, Lynch syndrome genes, BRIP1, RAD51C, or RAD51D.
👉Abrupt surgical menopause can adversely affect quality of life due to severe hot flashes, vaginal dryness, sexual dysfunction, sleep disturbances, and cognitive changes.
👉HRT should not be withheld for symptom control as short-term HRT does not seem to negate the risk-reducing effect of RRSO.
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