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  • Title: Congenital abnormalities and clinical features associated with Wilms' tumour: a comprehensive study from a centre serving a large population.
    Author: Ng A, Griffiths A, Cole T, Davison V, Griffiths M, Larkin S, Parkes SE, Mann JR, Grundy RG.
    Journal: Eur J Cancer; 2007 Jun; 43(9):1422-9. PubMed ID: 17499987.
    Abstract:
    Altogether 156 children treated for Wilms' tumour (WT) between 1970 and 1998 were studied. Sixty-six children, selected only by their attendance at clinic, were carefully examined and the findings compared to those from a case note review of 90 children. Congenital abnormalities were present in 45% of the examined cohort, in 19% of the case notes review group and in 30% overall. Novel findings included the association of WT with Marshall Smith syndrome, developmental delay in 3 of 4 cases of WT (one bilateral) and 1 sibling from consanguineous Pakistani families and another sibling also had leukaemia. The possibility of rare DNA repair or cancer predisposing disorders among these 4 families requires further study. Careful examination and history taking of an unselected patient cohort revealed a higher than expected incidence of clinical abnormalities which may be overlooked if not specifically sought.
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