Women with neurofibromatosis 1 are at a moderately increased risk of developing breast cancer and should be considered for early screening.
BACKGROUND
Malignancy risks in patients with neurofibromatosis 1 (NF1) are increased, but those occurring outside of the nervous system have not been clearly defined.


OBJECTIVE
To evaluate 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 women with NF1 aged >or=20 years was assessed and compared with population risks over the period 1975-2005 using a person-years-at-risk analysis.


RESULTS
There were 14 cases of breast cancers in the follow-up period, yielding a standardised incidence ratio (SIR) of 3.5 (95% CI 1.9 to 5.9). However, six breast cancers occurred in women in their 40s, and the SIR of breast cancer in women aged <50 years was 4.9 (95% CI 2.4 to 8.8).


CONCLUSIONS
Women with NF1 aged <50 years have a fivefold risk of breast cancer, are in the moderate risk category and should be considered for mammography from 40 years of age.
Sharif
S
S
Department of Clinical Genetics, Birmingham Women's Hospital, Birmingham, UK.
Moran
A
A
Huson
S M
SM
Iddenden
R
R
Shenton
A
A
Howard
E
E
Evans
D G R
DG
D017668:Age of Onset; D001943:Breast Neoplasms / Q000453:epidemiology* / Q000235:genetics*; D015331:Cohort Studies; D005260:Female; D005820:Genetic Testing / Q000379:methods*; D006801:Humans; D015994:Incidence; D008327:Mammography; D009456:Neurofibromatosis 1 / Q000235:genetics*; D009864:Ontario / Q000453:epidemiology; D012306:Risk