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Cancer incidence among persons with fragile X syndrome in Finland: a population-based study |
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| Authors: | Sund R Pukkala E Patja K |
| Affiliation: | ;National Research and Development Centre for Welfare and Health (STAKES), Helsinki, Finland ;Finnish Cancer Registry, Helsinki, Finland;University of Tampere, Tampere, Finland ;National Public Health Institute, Helsinki, Finland |
| Abstract: | Background Fragile X syndrome is a common inheritable cause of intellectual disability (ID) and is characterised by a large number of CGG repeats at the gene FMR1 located on the X-chromosome. It has been reported that this genetic mechanism may protect against malignant transformations. Methods We extracted from the Finnish registry on persons with ID a cohort of 302 persons with a fragile X diagnosis during 1982–1986. Follow-up for cancer incidence was performed in the Finnish Cancer Registry until the end of the year 2005. Results There were 11 reported cancers during the mean follow-up of 21.4 years per person. The expected number of cancers based on the average Finnish population was 13.8 and no statistically significant protective effect was detected [standardised incidence ratios (SIR) 0.80, confidence interval (CI) 95% 0.40–1.4]. An increased risk for lip cancer was found (SIR 23, CI 95% 2.8–85). Conclusions Confirmation of hypotheses about the mechanisms linking FXS and cancer needs further research. |
| Keywords: | cancer fragile X syndrome incidence intellectual disability |
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