Objective: Multiple endocrine neoplasia type 1 (MEN1) is associated with an early-onset elevated breast cancer risk. This finding potentially has implications for breast cancer screening for women with MEN1, and therefore it is necessary to assess whether other risk factors are involved to identify those at greatest risk.
Design: A cross-sectional case control study was performed using the Dutch MEN1 cohort, including >90% of the adult Dutch MEN1 population. All women with a confirmed MEN1 mutation received a questionnaire regarding cancer family history and breast cancer-related endocrine and general cancer risk factors.
Results: A total of 138 of 165 (84%) eligible women with MEN1 completed the questionnaire. Eleven of the 138 women had breast cancer. Another 34 relatives with breast cancer were identified in the families of the included women, of whom 11 were obligate MEN1 carriers, 14 had no MEN1 mutation, and 9 had an unknown MEN1 status. The median age at breast cancer diagnosis of women with MEN1 (n = 22) was 45 years (range, 30 to 80 years), in comparison with 57.5 years (range, 40 to 85 years) in female relatives without MEN1 (n = 14; P = 0.03) and 61.2 years in the Dutch reference population. Known endocrine risk factors and general risk factors were not different for women with and without breast cancer.
Conclusion: The increased breast cancer risk in MEN1 carriers was not related to other known breast cancer risk factors or familial cancer history, and therefore breast cancer surveillance from the age of 40 years for all women with MEN1 is justifiable.
- ENDOCRINE NEOPLASIA TYPE-1
- BRCA2 MUTATION CARRIERS
- SELECTION BIAS