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  1. Lidia Kasprzak, genetic counsellora,
  2. William D Foulkes (mdwf@musica.mcgill.ca), assistant professora,
  3. Andrew N Shelling, lecturer in reproductive science.b
  1. aDivision of Medical Genetics, Department of Medicine, McGill University, Montreal, Quebec, Canada H3G 1A4
  2. bResearch Centre in Reproductive Medicine, Department of Obstetrics and Gynaecology, National Women's Hospital, Auckland, New Zealand
  1. Correspondence to: Dr W D Foulkes, Department of Medicine, L10-116, Montreal General Hospital, 1650 Cedar Avenue, Montreal, Quebec, Canada H3G 1A4
  • Accepted 15 December 1998

Ovarian carcinoma is the fourth most common cause of cancer death in women in Western countries. Because of inadequate screening methods and the vague nature of the symptoms, patients present late in the course of the disease and the survival rate is poor. There are no strong environmental risk factors, and after age is controlled for the most important risk factor is a family history of ovarian carcinoma. The effect of a positive family history on a woman's risk of ovarian carcinoma is illustrated in table 1. In this review we focus on the hereditary aspects of ovarian carcinoma.

Summary points

  • Between 5% and 10% of cases of ovarian carcinoma are attributable to hereditary factors—mainly mutations in BRCA1 and BRCA2

  • The risk of ovarian carcinoma is 20%-50% up to age 70 years in carriers of a BRCA1 mutation, but much lower for women carrying a BRCA2 mutation outside exon 11

  • Mean age of onset is about 5 years younger in hereditary than in sporadic ovarian carcinoma

  • Serous adenocarcinoma is the most common histological type

  • The contraceptive pill protects against ovarian carcinoma, and prophylactic oophorectomy may help protect women at highest risk

  • The prognosis in hereditary and sporadic ovarian carcinoma seems to be similar

Methods

We reviewed original articles and expert reviews from journals cited in Medline between 1980 and 1998 and supplemented this information with unpublished data from our colleagues. We also included information from recently published books. In performing the Medline searches we used the following key words: hereditary ovarian carcinoma, breast and ovarian carcinoma syndrome, ovarian cancer screening, prophylactic oophorectomy.

Hereditary fraction of ovarian carcinoma

Between 5% and 10% of cases of ovarian carcinoma are the result of an inherited gene or genes. The percentage depends on the degree of relatives included in the calculation and the method of case ascertainment.2 Narod et al …

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