Elsevier

European Journal of Cancer

Volume 34, Issue 12, November 1998, Pages 1922-1926
European Journal of Cancer

Original Paper
Current policies for surveillance and management in women at risk of breast and ovarian cancer: a survey among 16 European family cancer clinics

https://doi.org/10.1016/S0959-8049(98)00288-3Get rights and content

Abstract

The recent isolation of breast cancer predisposing genes (BRCA1 and BRCA2) allows the identification of carriers within affected families. These carriers have a 50–85% risk of developing breast or ovarian cancer and need careful follow-up. The purpose of this study was to evaluate the management and screening protocols implemented in high risk families at various family cancer clinics in Europe. A questionnaire was mailed to the members of the European Familial Breast Cancer Collaborative Group (n=30) requesting information on the following issues: indication for surveillance of breasts and ovaries, the recommended protocol, coordination of the screening examination, prophylactic surgery, the specific management of breast cancer in a mutation carrier and the use of oestrogen. 16 centres from nine countries responded. Most centres recommend surveillance of the breasts if the lifetime risk exceeds 15–20%. The surveillance protocol that is generally advised comprises monthly self breast examination, examination by a specialist every 6 months and annual mammography, all starting from an age between 25 and 35 years. Surveillance of the ovaries is recommended in BRCA1 and BRCA2-mutation carriers, in members from breast/ovarian cancer families and in some centres in ‘breast cancer only’ families with an early onset of breast cancer. The recommended protocol includes gynaecological examination, sonography and estimation of CA-125 at yearly intervals starting from the age 30–35 years. Prophylactic mastectomy is considered for proven mutation carriers in some centres. Most centres consider prophylactic oophorectomy in mutation carriers and some centres also consider it for members of breast/ovarian cancer families. This survey provides insight into the guidelines for surveillance and management of familial breast cancer used at various family cancer clinics in Europe; this insight may contribute to the appropriate management of these high risk women. It should be emphasised that most recommendations are based on experts’ opinion rather than on any specific studies.

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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