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Women with Neurofibromatosis 1 (NF1) are at a moderately increased risk of developing breast cancer and should be considered for early screening
  1. Saba Sharif (saba.sharif{at}bwhct.nhs.uk)
  1. Birmingham Women's Hospital, United Kingdom
    1. Anthony Moran (tony.moran{at}nwcr.nhs.uk)
    1. North West Cancer Intelligence Service, United Kingdom
      1. Susan Huson (smhuson{at}aol.com)
      1. St Mary's Hospital, Manchester, United Kingdom
        1. Richard Iddenden (richard.iddenden{at}nwcr.nhs.uk)
        1. North West Cancer Intelligence Service, United Kingdom
          1. Andrew Shenton (andrew.shenton{at}cmmc.nhs.uk)
          1. St Mary's Hospital, Manchester, United Kingdom
            1. Elizabeth Howard (elizabeth.howard{at}cmmc.nhs.uk)
            1. St Mary's Hospital, Manchester, United Kingdom
              1. Gareth Evans (gareth.evans{at}cmmc.nhs.uk)
              1. St Mary's Manchester, United Kingdom

                Abstract

                Background: Malignancy risks in neurofibromatosis 1 (NF1) patients are elevated but those occurring outside of the nervous system have not been clearly defined. We evaluated the risk of breast cancer in women with NF1 in a population-based study.

                Methods: The risk of breast cancer in a cohort of 304 NF1 women aged ≥20 years was assessed and compared to population risks over the period 1975-2005 using a person years at risk analysis.

                Results: There were 14 breast cancers in the follow up period yielding a Standard Incidence Ratio (SIR) of 3.5 (95% CI 1.9-5.9). However, 6 breast cancers occurred in the forties and the SIR of breast cancer aged <50 years was 4.9 (95% CI 2.4-8.8).

                Interpretation: Women with NF1 have a five-fold risk of breast cancer <50 years of age, are in the moderate risk category and should be considered for mammography from 40 years of age.

                • BRCA1
                • NF1
                • breast cancer
                • screening

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