The IGF signalling pathway in Wilms tumours - A report from the ENCCA Renal Tumours Biology-driven drug development workshop

Mariana Maschietto, Jocelyn Charlton, Daniela Perotti, Paolo Radice, James I. Geller, Kathy Pritchard-Jones, Mark Weeks

Research output: Contribution to journalArticle

Abstract

It is hypothesised that Wilms tumour (WT) results from aberrant renal development due to its embryonic morphology, associated undifferentiated precursor lesions (termed nephrogenic rests) and embryonic kidney-like chromatin and gene expression profiles. From the study of overgrowth syndrome-associated WT, germline dysregulation was identified in the imprinted region at 11p15 affecting imprinted genes IGF2 and H19. This is also detected in ~70% sporadic cases, making this the most common somatic molecular aberration in WT. This review summarises the critical discussion at an international workshop held under the auspices of The European Network for Cancer Research in Children and Adolescents (ENCCA) consortium, where the potential for drug development to target IGF2 and the WT epigenome was debated. Here, we consider current cancer treatments which include targeting the IGF pathway and the use of methylation agents alone or in combination with other drugs in clinical trials of paediatric cancers. Finally, we discuss the possibility of the use of these drugs to treat patients with WT.

Original languageEnglish
Pages (from-to)8014-8026
Number of pages13
JournalOncotarget
Volume5
Issue number18
Publication statusPublished - 2014

Keywords

  • DNA methylation
  • IGF signalling pathway
  • IGF2
  • Targeted therapy
  • Wilms tumour

ASJC Scopus subject areas

  • Oncology

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    Maschietto, M., Charlton, J., Perotti, D., Radice, P., Geller, J. I., Pritchard-Jones, K., & Weeks, M. (2014). The IGF signalling pathway in Wilms tumours - A report from the ENCCA Renal Tumours Biology-driven drug development workshop. Oncotarget, 5(18), 8014-8026.