Wilms tumour (WT) is the most common paediatric kidney cancer and affects approximately one in 10 000 children. The tumour is associated with undifferentiated embryonic lesions called nephrogenic rests (NRs) or, when diffuse, nephroblastomatosis. WT or NRs can occur in both kidneys, termed bilateral disease, found in only 5–8% of cases. Management of bilateral WT presents a major clinical challenge in terms of maximising survival, preserving renal function and understanding underlying genetic risk. In this review, we compile clinical data from 545 published cases of bilateral WT and discuss recent progress in understanding the molecular basis of bilateral WT and its associated precursor NRs in the context of the latest radiological, surgical and epidemiological features.

中文翻译：

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双侧肾母细胞瘤：临床和分子特征的回顾


肾母细胞瘤 (WT) 是最常见的儿童肾癌，影响大约万分之一的儿童。该肿瘤与称为肾源性残留（NR）的未分化胚胎病变有关，或者当弥漫性时称为肾母细胞瘤病。 WT 或 NR 可发生在双肾，称为双侧疾病，仅见于 5-8% 的病例。双侧 WT 的管理在最大限度地提高生存率、保留肾功能和了解潜在的遗传风险方面提出了重大的临床挑战。在这篇综述中，我们汇编了 545 例已发表的双侧 WT 病例的临床数据，并在最新的放射学、手术和流行病学特征的背景下讨论了在了解双侧 WT 的分子基础及其相关前体 NR 方面的最新进展。