Hallmark discoveries in the biology of Wilms tumour

Daniela Perotti, Predictive Medicine: Molecular Bases of Genetic Risk, Department of Experimental Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Daniela.perotti@istitutotumori.mi.it.
Richard D. Williams, Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK.
Jenny Wegert, Theodor-Boveri-Institute/Biocenter, Developmental Biochemistry, Wuerzburg University, Wuerzburg, Germany.
Jack Brzezinski, Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada.
Mariana Maschietto, Research Center, Boldrini Children's Hospital, Campinas, São Paulo, Brazil.
Sara Ciceri, Predictive Medicine: Molecular Bases of Genetic Risk, Department of Experimental Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
David Gisselsson, Cancer Cell Evolution Unit, Division of Clinical Genetics, Department of Laboratory Medicine, Lund University, Lund, Sweden.
Samantha Gadd, Department of Pathology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Amy L. Walz, Division of Hematology,Oncology, Neuro-Oncology, and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Rhoikos Furtwaengler, Division of Pediatric Oncology and Hematology, Department of Pediatrics, Inselspital Bern University, Bern, Switzerland.
Jarno Drost, Princess Máxima Center for Paediatric Oncology, Utrecht, Netherlands.
Reem Al-Saadi, Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK.
Nicholas Evageliou, Divisions of Hematology and Oncology, Children's Hospital of Philadelphia, CHOP Specialty Care Center, Vorhees, NJ, USA.
Saskia L. Gooskens, Princess Máxima Center for Paediatric Oncology, Utrecht, Netherlands.
Andrew L. Hong, Aflac Cancer and Blood Disorders Center, Emory University and Children's Healthcare of Atlanta, Atlanta, GA, USA.
Andrew J. Murphy, Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN, USA.
Michael V. Ortiz, Department of Paediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Maureen J. O'Sullivan, Histology Laboratory, Children's Health Ireland at Crumlin, Dublin, Ireland.
Elizabeth A. Mullen, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MA, USA.
Marry M. van den Heuvel-Eibrink, Princess Máxima Center for Paediatric Oncology, Utrecht, Netherlands.
Conrad V. Fernandez, Division of Paediatric Hematology Oncology, IWK Health Centre and Dalhousie University, Halifax, Nova Scotia, Canada.
Norbert Graf, Department of Paediatric Oncology and Hematology, Saarland University Hospital, Homburg, Germany.
Paul E. Grundy, Department of Paediatrics Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
James I. Geller, Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
Jeffrey S. Dome, Division of Oncology, Center for Cancer and Blood Disorders, Children's National Hospital and the Department of Paediatrics, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
Elizabeth J. Perlman, Department of Pathology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Manfred Gessler, Theodor-Boveri-Institute/Biocenter, Developmental Biochemistry, Wuerzburg University, Wuerzburg, Germany.
Vicki Huff, Department of Genetics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Kathy Pritchard-Jones, Developmental Biology and Cancer Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK.

Document Type

Journal Article

Publication Date

10-17-2023

Journal

Nature reviews. Urology

DOI

10.1038/s41585-023-00824-0

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 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.

Department

Pediatrics

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