TY - JOUR
T1 - Congenital abnormalities and clinical features associated with Wilms’ tumour: A comprehensive study from a centre serving a large population
AU - Ng, A
AU - Cole, Trevor
AU - Davison, V
AU - Griffiths, Michael
AU - Larkin, S
AU - Parkes, SE
AU - Mann, JR
AU - Grundy, RG
AU - Griffiths, Annie
PY - 2007/6/1
Y1 - 2007/6/1
N2 - 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.
AB - 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.
U2 - 10.1016/j.ejca.2007.03.020
DO - 10.1016/j.ejca.2007.03.020
M3 - Article
C2 - 17499987
SN - 1879-0852
SN - 1879-2995
VL - 43
SP - 1422
EP - 1429
JO - European Journal of Cancer
JF - European Journal of Cancer
IS - 9
ER -