ABSTRACT
Heritable mutations in one of the BRCA genes cause approximately 8% to 13%
of epithelial ovarian cancers (1-3). Women with mutations in BRCA1 have a
35% to 60% chance of developing a BRCA-associated gynecologic (ovarian,
fallopian tube or primary peritoneal) cancer by age 70, corresponding to a rel-
ative risk of 30 to 45 times that of women in the general population (4-6).
Similarly, women with mutations in BRCA2 have a 10% to 27% chance of
developing a BRCA-associated gynecologic cancer by age 70, corresponding to a
relative risk of 6 to 20. Women with mutations in either of these genes are also at
tremendously increased risk of breast cancer, with 56% to 84% of mutation
carriers developing breast cancer by age 70 (4-7). Over the past decade, a great
deal has been learned about the efficacy of risk-reducing strategies in women
with an inherited predisposition secondary to a mutation in one of these genes.
Unfortunately, currently available ovarian cancer screening modalities have not
proven to be effective for women with an inherited risk of ovarian cancer. While
chemoprevention with oral contraceptives may reduce the risk of ovarian cancer,
both the incomplete prevention conferred against ovarian cancer as well as
possible deleterious impact on breast cancer risk limit their use as a risk-
reduction strategy in isolation. Given these issues, risk-reducing salpingo-
oophorectomy (RRSO) has become one of the cornerstones of risk reduction for
women with an inherited risk of ovarian cancer and should be considered in all
women who harbor a deleterious germline mutation in BRCA1 or BRCA2. In this
chapter, the sentinel studies supporting RRSO will be reviewed as well as the
salient issues surrounding both pre-and postoperative counseling.
