Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

Hallmark discoveries in the biology of Wilms tumour

Perotti, Daniela ; Williams, Richard D. ; Wegert, Jenny ; Brzezinski, Jack ; Maschietto, Mariana ; Ciceri, Sara ; Gisselsson, David LU ; Gadd, Samantha ; Walz, Amy L. and Furtwaengler, Rhoikos , et al. (2023) In Nature Reviews Urology
Abstract

The modern study of Wilms tumour was prompted nearly 50 years ago, when Alfred Knudson proposed the ‘two-hit’ model of tumour development. Since then, the efforts of researchers worldwide have substantially expanded our knowledge of Wilms tumour biology, including major advances in genetics — from cloning the first Wilms tumour gene to high-throughput studies that have revealed the genetic landscape of this tumour. These discoveries improve understanding of the embryonal origin of Wilms tumour, familial occurrences and associated syndromic conditions. Many efforts have been made to find and clinically apply prognostic biomarkers to Wilms tumour, for which outcomes are generally favourable, but treatment of some affected individuals... (More)

The modern study of Wilms tumour was prompted nearly 50 years ago, when Alfred Knudson proposed the ‘two-hit’ model of tumour development. Since then, the efforts of researchers worldwide have substantially expanded our knowledge of Wilms tumour biology, including major advances in genetics — from cloning the first Wilms tumour gene to high-throughput studies that have revealed the genetic landscape of this tumour. These discoveries improve understanding of the embryonal origin of Wilms tumour, familial occurrences and associated syndromic conditions. Many efforts have been made to find and clinically apply prognostic biomarkers to Wilms tumour, for which outcomes are generally favourable, but treatment of some affected individuals remains challenging. Challenges are also posed by the intratumoural heterogeneity of biomarkers. Furthermore, preclinical models of Wilms tumour, from cell lines to organoid cultures, have evolved. Despite these many achievements, much still remains to be discovered: further molecular understanding of relapse in Wilms tumour and of the multiple origins of bilateral Wilms tumour are two examples of areas under active investigation. International collaboration, especially when large tumour series are required to obtain robust data, will help to answer some of the remaining unresolved questions.

(Less)
Please use this url to cite or link to this publication:
author
; ; ; ; ; ; ; ; and , et al. (More)
; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; ; and (Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Nature Reviews Urology
publisher
Nature Publishing Group
external identifiers
  • pmid:37848532
  • scopus:85174232626
ISSN
1759-4812
DOI
10.1038/s41585-023-00824-0
language
English
LU publication?
yes
id
0003b748-72e6-4f7d-b4b7-54459ed6de5b
date added to LUP
2023-12-18 13:48:58
date last changed
2024-04-17 01:56:11
@article{0003b748-72e6-4f7d-b4b7-54459ed6de5b,
  abstract     = {{<p>The modern study of Wilms tumour was prompted nearly 50 years ago, when Alfred Knudson proposed the ‘two-hit’ model of tumour development. Since then, the efforts of researchers worldwide have substantially expanded our knowledge of Wilms tumour biology, including major advances in genetics — from cloning the first Wilms tumour gene to high-throughput studies that have revealed the genetic landscape of this tumour. These discoveries improve understanding of the embryonal origin of Wilms tumour, familial occurrences and associated syndromic conditions. Many efforts have been made to find and clinically apply prognostic biomarkers to Wilms tumour, for which outcomes are generally favourable, but treatment of some affected individuals remains challenging. Challenges are also posed by the intratumoural heterogeneity of biomarkers. Furthermore, preclinical models of Wilms tumour, from cell lines to organoid cultures, have evolved. Despite these many achievements, much still remains to be discovered: further molecular understanding of relapse in Wilms tumour and of the multiple origins of bilateral Wilms tumour are two examples of areas under active investigation. International collaboration, especially when large tumour series are required to obtain robust data, will help to answer some of the remaining unresolved questions.</p>}},
  author       = {{Perotti, Daniela and Williams, Richard D. and Wegert, Jenny and Brzezinski, Jack and Maschietto, Mariana and Ciceri, Sara and Gisselsson, David and Gadd, Samantha and Walz, Amy L. and Furtwaengler, Rhoikos and Drost, Jarno and Al-Saadi, Reem and Evageliou, Nicholas and Gooskens, Saskia L. and Hong, Andrew L. and Murphy, Andrew J. and Ortiz, Michael V. and O’Sullivan, Maureen J. and Mullen, Elizabeth A. and van den Heuvel-Eibrink, Marry M. and Fernandez, Conrad V. and Graf, Norbert and Grundy, Paul E. and Geller, James I. and Dome, Jeffrey S. and Perlman, Elizabeth J. and Gessler, Manfred and Huff, Vicki and Pritchard-Jones, Kathy}},
  issn         = {{1759-4812}},
  language     = {{eng}},
  month        = {{10}},
  publisher    = {{Nature Publishing Group}},
  series       = {{Nature Reviews Urology}},
  title        = {{Hallmark discoveries in the biology of Wilms tumour}},
  url          = {{http://dx.doi.org/10.1038/s41585-023-00824-0}},
  doi          = {{10.1038/s41585-023-00824-0}},
  year         = {{2023}},
}