Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop

Daniela Perotti; Peter Hohenstein; Italia Bongarzone; Mariana Maschietto; Mark Weeks; Paolo Radice; Kathy Pritchard-Jones

doi:10.1158/1535-7163.MCT-13-0335

Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop

Daniela Perotti, Peter Hohenstein, Italia Bongarzone, Mariana Maschietto, Mark Weeks, Paolo Radice, Kathy Pritchard-Jones^*

^*Corresponding author for this work

Royal (Dick) School of Veterinary Studies

Research output: Contribution to journal › Article › peer-review

Abstract

The European Network for Cancer Research in Children and Adolescents consortium organized a workshop in Rome, in June 2012, on "Biology-Driven Drug Development Renal Tumors Workshop" to discuss the current knowledge in pediatric renal cancers and to recommend directions for further research. Wilms tumor is the most common renal tumor of childhood and represents a success of pediatric oncology, with cure rates of more than 85% of cases. However, a substantial minority (∼25%) responds poorly to current therapies and requires "high-risk" treatment or relapse. Moreover, the successfully treated majority are vulnerable to the late effects of treatment, with nearly one quarter reporting severe chronic health conditions by 25 years of follow-up. Main purposes of this meeting were to advance our understanding on the molecular drivers in Wilms tumor, their heterogeneity and interdependencies; to provide updates on the clinical-pathologic associations with biomarkers; to identify eligible populations for targeted drugs; and to model opportunities to use preclinical model systems and prioritize targeted agents for early phase clinical trials. At least three different pathways are involved in Wilms tumor; this review represents the outcome of the workshop discussion on the WNT/β-catenin pathway in Wilms tumorigenesis. Mol Cancer Ther; 12(12); 2619-27. ©2013 AACR.

Original language	English
Pages (from-to)	2619-2627
Number of pages	9
Journal	Molecular Cancer Therapeutics
Volume	12
Issue number	12
DOIs	https://doi.org/10.1158/1535-7163.MCT-13-0335
Publication status	Published - Dec 2013

Keywords / Materials (for Non-textual outputs)

CANCER STEM-CELLS
BETA-CATENIN
KIDNEY DEVELOPMENT
SIGNALING PATHWAY
CTNNB1 MUTATIONS
EPITHELIAL TRANSFORMATION
NEPHRON DIFFERENTIATION
METANEPHRIC MESENCHYME
WT1 MUTATIONS
IN-VIVO

Access to Document

10.1158/1535-7163.MCT-13-0335

Is Wilms tumor a candidate neoplasia for treatment with WNT β-catenin pathway modulators
Published in final edited form as: Mol Cancer Ther. Dec 2013; 12(12): 10.1158/1535-7163.MCT-13-0335.
Accepted author manuscript, 579 KB

http://mct.aacrjournals.org/content/12/12/2619

Control of development and reproductive traits
Burdon, T. (Principal Investigator), Argyle, D. (Co-investigator), Ashworth, C. (Co-investigator), Beard, P. (Co-investigator), Brunton, P. (Co-investigator), Burt, D. (Co-investigator), Clinton, M. (Co-investigator), Dunn, I. (Co-investigator), Farquharson, C. (Co-investigator), Headon, D. (Co-investigator), Hocking, P. (Co-investigator), Hohenstein, P. (Co-investigator), Hume, D. (Co-investigator), Jackson, I. (Co-investigator), McColl, B. (Co-investigator), McGrew, M. (Co-investigator), McLachlan, G. (Co-investigator), Sang, H. (Co-investigator), Summers, K. (Co-investigator) & Whitelaw, B. (Co-investigator)
BBSRC
1/04/12 → 31/03/17
Project: Research

Cite this

Perotti, D., Hohenstein, P., Bongarzone, I., Maschietto, M., Weeks, M., Radice, P., & Pritchard-Jones, K. (2013). Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop. Molecular Cancer Therapeutics, 12(12), 2619-2627. https://doi.org/10.1158/1535-7163.MCT-13-0335

@article{749d29d10541409fa7ba36b6386495cb,

title = "Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop",

abstract = "The European Network for Cancer Research in Children and Adolescents consortium organized a workshop in Rome, in June 2012, on {"}Biology-Driven Drug Development Renal Tumors Workshop{"} to discuss the current knowledge in pediatric renal cancers and to recommend directions for further research. Wilms tumor is the most common renal tumor of childhood and represents a success of pediatric oncology, with cure rates of more than 85% of cases. However, a substantial minority (∼25%) responds poorly to current therapies and requires {"}high-risk{"} treatment or relapse. Moreover, the successfully treated majority are vulnerable to the late effects of treatment, with nearly one quarter reporting severe chronic health conditions by 25 years of follow-up. Main purposes of this meeting were to advance our understanding on the molecular drivers in Wilms tumor, their heterogeneity and interdependencies; to provide updates on the clinical-pathologic associations with biomarkers; to identify eligible populations for targeted drugs; and to model opportunities to use preclinical model systems and prioritize targeted agents for early phase clinical trials. At least three different pathways are involved in Wilms tumor; this review represents the outcome of the workshop discussion on the WNT/β-catenin pathway in Wilms tumorigenesis. Mol Cancer Ther; 12(12); 2619-27. {\textcopyright}2013 AACR.",

keywords = "CANCER STEM-CELLS, BETA-CATENIN, KIDNEY DEVELOPMENT, SIGNALING PATHWAY, CTNNB1 MUTATIONS, EPITHELIAL TRANSFORMATION, NEPHRON DIFFERENTIATION, METANEPHRIC MESENCHYME, WT1 MUTATIONS, IN-VIVO",

author = "Daniela Perotti and Peter Hohenstein and Italia Bongarzone and Mariana Maschietto and Mark Weeks and Paolo Radice and Kathy Pritchard-Jones",

year = "2013",

month = dec,

doi = "10.1158/1535-7163.MCT-13-0335",

language = "English",

volume = "12",

pages = "2619--2627",

journal = "Molecular Cancer Therapeutics",

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Perotti, D, Hohenstein, P, Bongarzone, I, Maschietto, M, Weeks, M, Radice, P & Pritchard-Jones, K 2013, 'Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop', Molecular Cancer Therapeutics, vol. 12, no. 12, pp. 2619-2627. https://doi.org/10.1158/1535-7163.MCT-13-0335

Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop. / Perotti, Daniela; Hohenstein, Peter; Bongarzone, Italia et al.
In: Molecular Cancer Therapeutics, Vol. 12, No. 12, 12.2013, p. 2619-2627.

Research output: Contribution to journal › Article › peer-review

TY - JOUR

T1 - Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop

AU - Perotti, Daniela

AU - Hohenstein, Peter

AU - Bongarzone, Italia

AU - Maschietto, Mariana

AU - Weeks, Mark

AU - Radice, Paolo

AU - Pritchard-Jones, Kathy

PY - 2013/12

Y1 - 2013/12

N2 - The European Network for Cancer Research in Children and Adolescents consortium organized a workshop in Rome, in June 2012, on "Biology-Driven Drug Development Renal Tumors Workshop" to discuss the current knowledge in pediatric renal cancers and to recommend directions for further research. Wilms tumor is the most common renal tumor of childhood and represents a success of pediatric oncology, with cure rates of more than 85% of cases. However, a substantial minority (∼25%) responds poorly to current therapies and requires "high-risk" treatment or relapse. Moreover, the successfully treated majority are vulnerable to the late effects of treatment, with nearly one quarter reporting severe chronic health conditions by 25 years of follow-up. Main purposes of this meeting were to advance our understanding on the molecular drivers in Wilms tumor, their heterogeneity and interdependencies; to provide updates on the clinical-pathologic associations with biomarkers; to identify eligible populations for targeted drugs; and to model opportunities to use preclinical model systems and prioritize targeted agents for early phase clinical trials. At least three different pathways are involved in Wilms tumor; this review represents the outcome of the workshop discussion on the WNT/β-catenin pathway in Wilms tumorigenesis. Mol Cancer Ther; 12(12); 2619-27. ©2013 AACR.

AB - The European Network for Cancer Research in Children and Adolescents consortium organized a workshop in Rome, in June 2012, on "Biology-Driven Drug Development Renal Tumors Workshop" to discuss the current knowledge in pediatric renal cancers and to recommend directions for further research. Wilms tumor is the most common renal tumor of childhood and represents a success of pediatric oncology, with cure rates of more than 85% of cases. However, a substantial minority (∼25%) responds poorly to current therapies and requires "high-risk" treatment or relapse. Moreover, the successfully treated majority are vulnerable to the late effects of treatment, with nearly one quarter reporting severe chronic health conditions by 25 years of follow-up. Main purposes of this meeting were to advance our understanding on the molecular drivers in Wilms tumor, their heterogeneity and interdependencies; to provide updates on the clinical-pathologic associations with biomarkers; to identify eligible populations for targeted drugs; and to model opportunities to use preclinical model systems and prioritize targeted agents for early phase clinical trials. At least three different pathways are involved in Wilms tumor; this review represents the outcome of the workshop discussion on the WNT/β-catenin pathway in Wilms tumorigenesis. Mol Cancer Ther; 12(12); 2619-27. ©2013 AACR.

KW - CANCER STEM-CELLS

KW - BETA-CATENIN

KW - KIDNEY DEVELOPMENT

KW - SIGNALING PATHWAY

KW - CTNNB1 MUTATIONS

KW - EPITHELIAL TRANSFORMATION

KW - NEPHRON DIFFERENTIATION

KW - METANEPHRIC MESENCHYME

KW - WT1 MUTATIONS

KW - IN-VIVO

U2 - 10.1158/1535-7163.MCT-13-0335

DO - 10.1158/1535-7163.MCT-13-0335

M3 - Article

C2 - 24258344

SN - 1535-7163

VL - 12

SP - 2619

EP - 2627

JO - Molecular Cancer Therapeutics

JF - Molecular Cancer Therapeutics

IS - 12

ER -

Is Wilms Tumor a Candidate Neoplasia for Treatment with WNT/β-Catenin Pathway Modulators?-A Report from the Renal Tumors Biology-Driven Drug Development Workshop

Abstract

Keywords / Materials (for Non-textual outputs)

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Projects

Control of development and reproductive traits

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