title: Pathology, genetics and cytogenetics of Wilms' tumour.
abstract: Wilms' tumour (WT) is an embryonal cancer of childhood and is thought to be derived from embryonic kidney precursor cells. The Knudson two hit model was initially thought to occur in WT, but findings emerging from genetic and cytogenetic studies in the past two decades have implicated several genetic events. Recent techniques in genetic analysis have improved our ability to characterise changes in genes involved in WT which include WT1, CTNNB1, IGF2 and WTX. These genetic events have not only provided insight into the pathobiology of this malignancy, but the recognition of these candidate genes may offer potential targets for novel therapies. In this review, we will provide an overview of the pathological, genetic and cytogenetic characteristics of WT.
authors.affiliation: School of Pathology and Laboratory Medicine, University of Western Australia, QEII Medical Centre, Nedlands, Western Australia, Australia.
authors.forename: Reena
authors.initials: R
authors.lastname: Md Zin
authors.forename: Ashleigh
authors.initials: A
authors.lastname: Murch
authors.forename: Adrian
authors.initials: A
authors.lastname: Charles
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003582:Cytogenetics; D016162:Genes, Wilms Tumor; D006801:Humans; D007223:Infant; D007335:Insulin-Like Growth Factor II / Q000235:genetics; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology*; D025521:Tumor Suppressor Proteins / Q000235:genetics; D025721:WT1 Proteins / Q000235:genetics; D009396:Wilms Tumor / Q000235:genetics / Q000473:pathology*; D051176:beta Catenin / Q000235:genetics
chemical_list: C517118:AMER1 protein, human; D048868:Adaptor Proteins, Signal Transducing; C495270:CTNNB1 protein, human; D025521:Tumor Suppressor Proteins; D025721:WT1 Proteins; D051176:beta Catenin; D007335:Insulin-Like Growth Factor II