{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,29]],"date-time":"2026-03-29T00:03:33Z","timestamp":1774742613869,"version":"3.50.1"},"reference-count":49,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[1994,1,1]],"date-time":"1994-01-01T00:00:00Z","timestamp":757382400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[1994,1,1]],"date-time":"1994-01-01T00:00:00Z","timestamp":757382400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[1994,1,1]],"date-time":"1994-01-01T00:00:00Z","timestamp":757382400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["World j. surg."],"published-print":{"date-parts":[[1994,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hereditary breast cancer (HBC) shows extant clinical and genetic heterogeneity. Clinically one finds the onset of breast cancer at an early age, an excess of bilaterality, and patterns of multiple primary cancer such as combinations of breast and ovarian carcinoma in the hereditary breast\u2010ovarian cancer (HBOC) syndrome. In addition to HBOC, one sees a variety of putative breast cancer\u2010prone genotypes inclusive of hereditary site\u2010specific breast cancer, and the Li\u2010Frameni (SBLA) syndrome that is characterized by cancers involving all three germinal layers including sarcomas, brain tumors, leukemia, lymphoma, and adrenal cortical carcinoma in addition to often markedly early\u2010onset breast cancer. Breast cancer is also associated with autosomal dominantly inherited Cowden's disease and autosomal recessively inherited ataxia\u2010telangiectasia. Examples of pedigrees depicting clincal examples of these several HBC syndromes are presented in order to describe HBC's heterogeneity. The recent identification of the BRCA1 gene in early\u2010onset hereditary sitespecific breast cancer and the HBOC syndrome has led to new challenges for the genetic counselor. We review genetic counseling, which embraces surveillance and management recommendations that are responsive to the natural history of HBC and address the concept for future development of centers of expertise for HBC in the interest of improving cancer control.<\/jats:p>","DOI":"10.1007\/bf00348188","type":"journal-article","created":{"date-parts":[[2004,12,10]],"date-time":"2004-12-10T07:37:32Z","timestamp":1102664252000},"page":"21-31","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":35,"title":["Hereditary breast cancer and family cancer syndromes"],"prefix":"10.1002","volume":"18","author":[{"given":"Henry T.","family":"Lynch","sequence":"first","affiliation":[{"name":"Department of Preventive Medicine and Public Health Creighton University School of Medicine 2500 California Plaza 8178 Omaha Nebraska USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jane","family":"Lynch","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine and Public Health Creighton University School of Medicine 2500 California Plaza 8178 Omaha Nebraska USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Theresa","family":"Conway","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine and Public Health Creighton University School of Medicine 2500 California Plaza 8178 Omaha Nebraska USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrice","family":"Watson","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine and Public Health Creighton University School of Medicine 2500 California Plaza 8178 Omaha Nebraska USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean","family":"Feunteum","sequence":"additional","affiliation":[{"name":"Laboratoire D'Oncologie Moleculaire Institut Gustave\u2010Roussy 94805 Villejuif Cedex France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gilbert","family":"Lenoir","sequence":"additional","affiliation":[{"name":"International Agency for Research on Cancer 150, Cours Albert Thomas 69372 Lyon Cedex 08 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven","family":"Narod","sequence":"additional","affiliation":[{"name":"Montreal General Hospital 1650 Cedar Avenue Montreal Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr.","given":"Robert","family":"Fitzgibbons","sequence":"additional","affiliation":[{"name":"Department of General Surgery Creighton University School of Medicine 601\u00a0N. 30th 68131 Omaha Nebraska USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1994,1]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19920915)70:4 <1653::AID-CNCR2820701603>3.0.CO;2-1"},{"key":"e_1_2_1_3_1","volume-title":"Trait\u00e9 des Tumeurs. 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