Hallmark discoveries in the biology of Wilms tumour.

Perotti, Daniela; Williams, Richard D; Wegert, Jenny; Brzezinski, Jack; Maschietto, Mariana; Ciceri, Sara; Gisselsson, David; Gadd, Samantha; Walz, Amy L; Furtwängler, Rhoikos; Drost, Jarno; Al-Saadi, Reem; Evageliou, Nicholas; Gooskens, Saskia L; Hong, Andrew L; Murphy, Andrew J; Ortiz, Michael V; O'Sullivan, Maureen J; Mullen, Elizabeth A; van den Heuvel-Eibrink, Marry M; ... (2024). Hallmark discoveries in the biology of Wilms tumour. Nature Reviews. Urology, 21(3), pp. 158-180. Nature Publishing Group 10.1038/s41585-023-00824-0

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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.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Haematology/Oncology

UniBE Contributor:

Furtwängler, Rhoikos

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1759-4820

Publisher:

Nature Publishing Group

Language:

English

Submitter:

Pubmed Import

Date Deposited:

18 Oct 2023 10:28

Last Modified:

08 Mar 2024 00:13

Publisher DOI:

10.1038/s41585-023-00824-0

PubMed ID:

37848532

URI:

https://boris.unibe.ch/id/eprint/187272

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