{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T19:29:40Z","timestamp":1772825380084,"version":"3.50.1"},"reference-count":22,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2002,8,21]],"date-time":"2002-08-21T00:00:00Z","timestamp":1029888000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Intl Journal of Cancer"],"published-print":{"date-parts":[[2002,10,20]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Dedicated clinics have been established for the early diagnosis and treatment of women at risk for inherited breast cancer, but the effects of such interventions are currently unproven. This second report on prospectively diagnosed inherited breast cancer from the European collaborating centres supports the previous conclusions and adds information on genetic heterogeneity and the effect of oophorectomy. Of 249 patients, 20% had carcinoma <jats:italic>in situ<\/jats:italic> (CIS), 54% had infiltrating cancer without spread (CaN0) and 26% had cancer with spread (CaN+). Five\u2010year survival was 100% for CIS, 94% for CaN0 and 72% for CaN+ (<jats:italic>p<\/jats:italic> = 0.007). Thirty\u2010six patients had <jats:italic>BRCA1<\/jats:italic> mutations, and 8 had <jats:italic>BRCA2<\/jats:italic> mutations. Presence of <jats:italic>BRCA1<\/jats:italic> mutation was associated with infiltrating cancer, high grade and lack of oestrogen receptor (<jats:italic>p<\/jats:italic> &lt; 0.05 for all 3 characteristics). For <jats:italic>BRCA1<\/jats:italic> mutation carriers, 5\u2010year survival was 63% <jats:italic>vs.<\/jats:italic> 91% for noncarriers (<jats:italic>p<\/jats:italic> = 0.04). For CaN0 patients, mutation carriers had 75% 5\u2010year disease\u2010free survival <jats:italic>vs.<\/jats:italic> 96% for noncarriers (<jats:italic>p<\/jats:italic> = 0.01). Twenty\u2010one of the mutation carriers had undergone prophylactic oophorectomy, prior to or within 6 months of diagnosis in 13 cases. All but 1 relapse occurred in the 15 who had kept their ovaries, (<jats:italic>p<\/jats:italic> &lt; 0.01); no relapse occurred in those who had removed the ovaries within 6 months (<jats:italic>p<\/jats:italic> = 0.04) Contralateral cancer was more frequently observed in mutation noncarriers, but this finding did not reach statistical significance. Our findings support the concept that <jats:italic>BRCA1<\/jats:italic> cancer is biologically different from other inherited breast cancers. While current screening protocols appear satisfactory for the majority of women at risk of familial breast cancer, this may not be the case for <jats:italic>BRCA1<\/jats:italic> mutation carriers. The observed effect of oophorectomy was striking. \u00a9 2002 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ijc.10641","type":"journal-article","created":{"date-parts":[[2002,10,9]],"date-time":"2002-10-09T02:28:47Z","timestamp":1034130527000},"page":"555-559","source":"Crossref","is-referenced-by-count":94,"title":["Survival in prospectively ascertained familial breast cancer: Analysis of a series stratified by tumour characteristics, <i>BRCA<\/i> mutations and oophorectomy"],"prefix":"10.1002","volume":"101","author":[{"given":"P\u00e5l","family":"M\u00f8ller","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c5ke","family":"Borg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. 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