Original PaperCurrent policies for surveillance and management in women at risk of breast and ovarian cancer: a survey among 16 European family cancer clinics
Introduction
Approximately 5% of breast cancer is inherited as an autosomal dominant trait[1]. Since the identification of genes (BRCA1 and BRCA2) which predispose to breast or ovarian cancer, more than 200 different mutations have been reported2, 3, 4, 5. Although reports show wide variation in the risk that carriers of these mutations will develop breast cancer, most studies indicate that the lifetime risk is more than 50%6, 7, 8, 9, 10. The risk of developing ovarian cancer differs between carriers of a BRCA1 mutation (risk at age 70 years: 44%)[6]and those carrying a BRCA2 mutation (risk at age 70 years: 27%)[11]. Studies also indicate that the risk of ovarian cancer is associated with the site of the mutation within the respective genes12, 13. Because of these strongly increased risks, women carrying such genes need careful follow up. Unfortunately, the current available techniques of early detection of breast and ovarian cancer are far from ideal. For this reason, such mutilating options as prophylactic mastectomy might be considered in some cases.
The aim of the present study was to evaluate the guidelines followed at all relevant European centres for surveillance and management of high risk women. It is hoped that the results of this assessment will lead to the establishment of preliminary recommendations based on best practice and expert opinion.
Section snippets
Materials and methods
In 1996 a European collaborative group of clinicians specialising in familial breast cancer was established, the European Familial Breast Cancer Collaborative Group. All these specialists were members of the Breast Cancer Linkage Consortium, a collaborative group that played an important role in the identification of the genes involved in familial breast cancer. The members, who are mainly clinical geneticists and oncologists, are directly involved in the care of breast cancer families at
Results
Completed questionnaires were received from 16 centres in 9 countries (Sweden, Norway, U.K., Italy, Germany, Finland, France, Denmark and The Netherlands). With two exceptions, all centres recommended surveillance of the breasts if the lifetime risk of developing breast cancer is more than double the population risk (approximately 15%). The protocol recommended in most centres includes monthly self breast palpation, palpation by a physician every 6 months and annual mammography (Table 1).
Discussion
During the last 5 years, a large number of family cancer clinics have been established all over Europe. These multidisciplinary clinics are specialised in the counselling and genetic testing of those families that are at increased risk of developing cancer. Due both to the relatively high estimated prevalence of BRCA-1 and -2 mutations in the general population and also to the ample attention paid by the media to familial breast cancer, most relatives who visit such clinics are from breast
Conclusions
This report presents the results of an evaluation of the protocol used in the various centres for the surveillance and management of breast cancer families. The results reveal agreement on several parts of the protocol, but also conspicuous differences of opinion on other parts. It should be emphasised that most recommendations do not have a strong scientific basis and that future studies are urgently needed. Studies that might preferably be conducted on a European basis are an evaluation of
Acknowledgements
We would like to thank our colleagues who were so kind to complete the questionnaires: H. Olssen, B. Milner, M. Montagna, M.W. Beckmann, Y.J. Bignon, A.M. Gerdes, I. Eisinger and L. Beex.
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