Wilms tumour

Definition

Wilm's tumour is a predominantly paediatric, malignant tumour of the kidney (nephroblastoma). It generally presents unilaterally, and is bilateral in less than 5% of patients. Pathologically, three cell types can be involved: blastemal, stromal and epithelial cells.

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Latest Research and Reviews

  • Research |

    Elizabeth Perlman and colleagues use genome-wide sequencing, RNA expression, DNA copy number and methylation analyses to characterize the genomic landscape of Wilms tumors. Their integrated analyses implicate two major classes of genetic changes in Wilms tumors that preserve the progenitor state and/or interrupt normal kidney development.

    • Samantha Gadd
    • , Vicki Huff
    • , Amy L Walz
    • , Ariadne H A G Ooms
    • , Amy E Armstrong
    • , Daniela S Gerhard
    • , Malcolm A Smith
    • , Jaime M Guidry Auvil
    • , Daoud Meerzaman
    • , Qing-Rong Chen
    • , Chih Hao Hsu
    • , Chunhua Yan
    • , Cu Nguyen
    • , Ying Hu
    • , Leandro C Hermida
    • , Tanja Davidsen
    • , Patee Gesuwan
    • , Yussanne Ma
    • , Zusheng Zong
    • , Andrew J Mungall
    • , Richard A Moore
    • , Marco A Marra
    • , Jeffrey S Dome
    • , Charles G Mullighan
    • , Jing Ma
    • , David A Wheeler
    • , Oliver A Hampton
    • , Nicole Ross
    • , Julie M Gastier-Foster
    • , Stefan T Arold
    •  & Elizabeth J Perlman
    Nature Genetics 49, 1487–1494
  • Research |

    Nazneen Rahman, Geert Kops and colleagues report the identification of biallelic loss-of-function mutations in TRIP13 in six individuals with Wilms tumor who presented with features of mosaic variegated aneuploidy. They show that TRIP13-mutant cells show spindle assembly checkpoint defects and suggest that mechanisms leading to aneuploidy may contribute directly to increased cancer risk.

    • Shawn Yost
    • , Bas de Wolf
    • , Sandra Hanks
    • , Anna Zachariou
    • , Chiara Marcozzi
    • , Matthew Clarke
    • , Richarda M de Voer
    • , Banafsheh Etemad
    • , Esther Uijttewaal
    • , Emma Ramsay
    • , Harriet Wylie
    • , Anna Elliott
    • , Susan Picton
    • , Audrey Smith
    • , Sarah Smithson
    • , Sheila Seal
    • , Elise Ruark
    • , Gunnar Houge
    • , Jonathon Pines
    • , Geert J P L Kops
    •  & Nazneen Rahman
    Nature Genetics 49, 1148–1151
  • Research |

    Nazneen Rahman and colleagues identify inactivating germline mutations in the gene encoding the transcriptional repressor REST in familial and non-familial cases of Wilms tumor. The mutations cluster in the DNA-binding domain of REST and compromise REST transcriptional repression.

    • Shazia S Mahamdallie
    • , Sandra Hanks
    • , Kristen L Karlin
    • , Anna Zachariou
    • , Elizabeth R Perdeaux
    • , Elise Ruark
    • , Chad A Shaw
    • , Alexander Renwick
    • , Emma Ramsay
    • , Shawn Yost
    • , Anna Elliott
    • , Jillian Birch
    • , Michael Capra
    • , Juliet Gray
    • , Juliet Hale
    • , Judith Kingston
    • , Gill Levitt
    • , Thomas McLean
    • , Eamonn Sheridan
    • , Anthony Renwick
    • , Sheila Seal
    • , Charles Stiller
    • , Neil Sebire
    • , Thomas F Westbrook
    •  & Nazneen Rahman
    Nature Genetics 47, 1471–1474
  • Reviews |

    The microRNA (miRNA) biogenesis pathway is frequently altered in cancer, leading to global downregulation of miRNA levels in some cancer types. This Review discusses the alterations that affect miRNA biogenesis in cancer.

    • Shuibin Lin
    •  & Richard I. Gregory
    Nature Reviews Cancer 15, 321–333
  • Research | | open

    Wilms tumour is a rare renal neoplasm that primarily affects children but the genomic changes responsible for its development are currently largely unknown. In this study, the authors identify somatic mutations of the MLLT1 gene that are potentially involved in the aetiology of a subset of Wilms tumours.

    • Elizabeth J. Perlman
    • , Samantha Gadd
    • , Stefan T. Arold
    • , Anand Radhakrishnan
    • , Daniela S. Gerhard
    • , Lawrence Jennings
    • , Vicki Huff
    • , Jaime M. Guidry Auvil
    • , Tanja M. Davidsen
    • , Jeffrey S. Dome
    • , Daoud Meerzaman
    • , Chih Hao Hsu
    • , Cu Nguyen
    • , James Anderson
    • , Yussanne Ma
    • , Andrew J. Mungall
    • , Richard A. Moore
    • , Marco A. Marra
    • , Charles G. Mullighan
    • , Jing Ma
    • , David A. Wheeler
    • , Oliver A. Hampton
    • , Julie M. Gastier-Foster
    • , Nicole Ross
    •  & Malcolm A. Smith

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