title: Risk of ovarian cancer in BRCA1 and BRCA2 mutation-negative hereditary breast cancer families.
abstract: Women from site-specific hereditary breast cancer families who carry a BRCA1 or BRCA2 mutation are at increased risk for ovarian cancer. It is less clear, however, whether individuals from hereditary breast cancer families who do not carry such a mutation are also at increased ovarian cancer risk. To determine whether women from BRCA mutation-negative hereditary breast cancer families are at increased risk for ovarian cancer, 199 probands from BRCA mutation-negative, site-specific breast cancer kindreds who consented to prospective follow-up at the time of genetic testing were identified. The incidence of new breast and ovarian cancers in probands and their families since receipt of their genetic test results was determined by questionnaire. The expected number of cancers and standardized incidence ratios (SIRs) were determined from age-specific cancer incidence rates from the Surveillance, Epidemiology, and End Results (SEER) program by using the method of Byar. All statistical tests were two-sided. During 2534 women-years of follow-up in 165 kindreds, 19 new cases of breast cancer were diagnosed, whereas only 6.07 were expected (SIR = 3.13, 95% confidence interval [CI] = 1.88 to 4.89; P < .001), and one case of ovarian cancer was diagnosed, whereas only 0.66 was expected (SIR = 1.52, 95% CI = 0.02 to 8.46; P = .48). These results suggest that women from BRCA mutation-negative, site-specific breast cancer families are not at increased risk for ovarian cancer.
authors.affiliation: Department of Gynecology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
authors.forename: Noah D
authors.initials: ND
authors.lastname: Kauff
authors.forename: Nandita
authors.initials: N
authors.lastname: Mitra
authors.forename: Mark E
authors.initials: ME
authors.lastname: Robson
authors.forename: Karen E
authors.initials: KE
authors.lastname: Hurley
authors.forename: Shaokun
authors.initials: S
authors.lastname: Chuai
authors.forename: Deborah
authors.initials: D
authors.lastname: Goldfrank
authors.forename: Eve
authors.initials: E
authors.lastname: Wadsworth
authors.forename: Johanna
authors.initials: J
authors.lastname: Lee
authors.forename: Tessa
authors.initials: T
authors.lastname: Cigler
authors.forename: Patrick I
authors.initials: PI
authors.lastname: Borgen
authors.forename: Larry
authors.initials: L
authors.lastname: Norton
authors.forename: Richard R
authors.initials: RR
authors.lastname: Barakat
authors.forename: Kenneth
authors.initials: K
authors.lastname: Offit
mesh_terms: D000328:Adult; D000368:Aged; D001943:Breast Neoplasms / Q000235:genetics*; D016001:Confidence Intervals; D005260:Female; D005500:Follow-Up Studies; D019398:Genes, BRCA1; D024522:Genes, BRCA2; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D015994:Incidence; D007585:Jews / Q000235:genetics / Q000706:statistics & numerical data; D008875:Middle Aged; D009154:Mutation; D016017:Odds Ratio; D010051:Ovarian Neoplasms / Q000453:epidemiology* / Q000235:genetics; D011446:Prospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D018426:SEER Program; D011795:Surveys and Questionnaires; D014481:United States / Q000453:epidemiology