School of Pathology and Laboratory Medicine, University of Western Australia, QEII Medical Centre, Nedlands, Western Australia, Australia.
Pathology. 2011 Jun;43(4):302-12. doi: 10.1097/PAT.0b013e3283463575.
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.
威尔姆斯瘤（WT）是一种儿童胚胎性癌，被认为来源于胚胎肾前体细胞。最初认为 Knudson 双打击模型发生在 WT 中，但过去二十年的遗传和细胞遗传学研究结果表明存在几种遗传事件。最近的遗传分析技术提高了我们描述 WT 相关基因（包括 WT1、CTNNB1、IGF2 和 WTX）变化的能力。这些遗传事件不仅为该恶性肿瘤的病理生物学提供了深入了解，而且这些候选基因的识别可能为新的治疗方法提供潜在的靶点。在这篇综述中，我们将概述 WT 的病理、遗传和细胞遗传学特征。
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