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Case History

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Image not available. A 36-year-old woman has a history of familial cancer including her mother and a sister, both with ovarian cancer diagnosed at age 57 and 46 years, respectively, and an aunt on her mother's side with breast cancer diagnosed at age 37 years. She asks you for advice with regard to prophylactic surgery to decrease her risk of developing ovarian cancer.

Who should be offered a prophylactic salpingo-oophorectomy?

What is the optimal timing for a prophylactic salpingo-oophorectomy?

How should a prophylactic salpingo-oophorectomy be performed?

How do you manage women following risk-reducing salpingo-oophorectomy?

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Background

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Who should be offered a prophylactic salpingo-oophorectomy?

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Image not available. Bilateral salpingo-oophorectomy is an option for reducing the risk of ovarian cancer in women with hereditary predisposition for this malignancy. Familial or inherited syndromes account for approximately 10% of cases of ovarian cancer. Familial cancer syndromes associated with ovarian cancer are the (a) breast–ovarian cancer syndrome, and (b) hereditary non-polyposis colorectal cancer syndrome (HNPCC).

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The most common hereditary syndrome is the breast–ovarian cancer syndrome which is caused by germline mutations of the tumour suppressor genes BRCA1 and BRCA2. BRCA1 mutation carriers have an up to 60% lifetime risk of ovarian cancer and an up to 80% lifetime risk of breast cancer. The lifetime risk for BRCA2 mutation carriers to develop ovarian cancer is up to 40%, the risk for breast cancer up to 80%. In addition, BRCA mutations predispose for the development of fallopian tube and primary peritoneal carcinomas and might be associated with serous papillary carcinomas of the endometrium.

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Women with documented BRCA1 or BRCA2 mutations who undergo risk-reducing salpingo-oophorectomy have a 80%–96% reduction in the risk of ovarian and fallopian tube cancer.1,2 Furthermore, if the operation is performed before the menopause, it is associated with a 50%–68% reduction in the risk of developing breast cancer. Therefore, there is clear evidence to support offering risk-reducing salpingo-oophorectomy to women with known mutations in BRCA1 or BRCA2.3

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What is the optimal timing for a prophylactic salpingo-oophorectomy?

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For women with BRCA1 mutations, the risk of ovarian cancer begins to rise in the late 30s and early 40s; for women with BRCA2 mutations, approximately ten years later. For BRCA1 mutation carriers, risk-reducing salpingo-oophorectomy should therefore be offered after completion of childbearing at age 35 to 40 years. Although the risk of ovarian cancer for women with BRCA2 mutation is only up to 3% by age 50 years, the risk of breast cancer at this age may be as high as 34%. To postpone risk-reducing salpingooophorectomy until menopause may therefore cause a substantial loss of protection against breast cancer which prophylactic surgery provides.3 Therefore a prophylactic salpingo-oophorectomy for a BRCA2 mutation carrier is advisable at age 40 to 45 years.

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How should a prophylactic salpingo-oophorectomy be performed?

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