title: Educational review: role of the surgeon in hereditary breast cancer.
abstract: Up to 10% of the breast cancers detected in the United States are related to an inherited germline mutation, usually in the BRCA1 or BRCA2 genes, and the majority of these patients will at some point require surgical evaluation and/or treatment. Women who harbor a genetic predisposition for breast cancer face an increased risk for early onset disease, bilateral tumors, and other non-breast malignancies, such as ovarian cancer. These issues raise questions regarding the appropriate surveillance regimen, and the potential efficacy of risk reduction strategies that should be considered. Once a breast cancer diagnosis has been established, the prognosis appears to be similar to stage-controlled sporadic breast cancer, despite an increased prevalence of adverse primary tumor features. However, the role of breast conservation therapy for these patients and the optimal means of addressing the substantially increased risk for contralateral tumors is not yet defined. The reported literature in this area, including a discussion of the value of genetic counseling and genetic testing, is reviewed.
authors.affiliation: Department of Surgical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston 77030, USA.
authors.forename: L A
authors.initials: LA
authors.lastname: Newman
authors.forename: H M
authors.initials: HM
authors.lastname: Kuerer
authors.forename: K K
authors.initials: KK
authors.lastname: Hunt
authors.forename: G
authors.initials: G
authors.lastname: Vlastos
authors.forename: F C
authors.initials: FC
authors.lastname: Ames
authors.forename: M I
authors.initials: MI
authors.lastname: Ross
authors.forename: S E
authors.initials: SE
authors.lastname: Singletary
mesh_terms: D000328:Adult; D000368:Aged; D024682:BRCA2 Protein; D001943:Breast Neoplasms / Q000235:genetics* / Q000517:prevention & control / Q000601:surgery*; D018890:Chemoprevention; D005260:Female; D019398:Genes, BRCA1; D005817:Genetic Counseling; D005820:Genetic Testing; D006801:Humans; D008019:Life Style; D008408:Mastectomy; D008875:Middle Aged; D009363:Neoplasm Proteins; D010051:Ovarian Neoplasms / Q000235:genetics / Q000517:prevention & control / Q000601:surgery; D010052:Ovariectomy; D010375:Pedigree; D011379:Prognosis; D012307:Risk Factors; D014157:Transcription Factors
chemical_list: D024682:BRCA2 Protein; D009363:Neoplasm Proteins; D014157:Transcription Factors