Predisposition Genes
The gene spectrum in this cohort emphasized organ-specific and developmental syndrome genes rather than the DNA repair genes often seen in prior pediatric cancer cohorts. WT1-associated Wilms tumours in the follow-up group arose from trunc…
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The gene spectrum in this cohort emphasized organ-specific and developmental syndrome genes rather than the DNA repair genes often seen in prior pediatric cancer cohorts. WT1-associated Wilms tumours in the follow-up group arose from truncating variants. RB1 showed the highest penetrance for retinoblastoma among dominantly inherited genes in the study. WT1 showed lower penetrance for Wilms tumour than RB1 did for retinoblastoma.