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Childhood solid tumours: a developmental disorder

Nature Reviews Cancer volume 5, pages 481–488 (2005)Cite this article

Abstract

Several lines of evidence demonstrate that the biology, genetics and environment of childhood solid tumours (CSTs) sets them apart from adult solid tumours. The nature of the progenitor cells from which these tumours arise, and their immature tissue environment, allows CSTs to develop with fewer defects in cell regulatory processes than adult cancers. These differences could explain why CSTs are more susceptible to therapeutic intervention than adult tumours. How does the aetiology of these cancers differ from those occurring in adults and how might this affect the development of more effective therapies?

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Figure 1: Tumour incidence with age.
Figure 2: WNT and SHH signalling pathways.
Figure 3: The WT1 gene in metanephros and Wilms' tumour development.

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Acknowledgements

P.J.S. and D.A.W. are supported by the Nottingham Children's Brain Tumour Research Centre. Thanks to J. Hewitt, B. Coyle, V. Sottile and R. Grundy for their constructive criticism of the manuscript. Thanks also to M. Hyatt for help with the production of figure 1 and J. Davies for figure 3.

Author information

Authors and Affiliations

  1. the Children's Brain Tumour Research Centre, Institute of Genetics, University of Nottingham, Queen's Medical Centre, Nottingham, NG7 2UH, UK

    Paul J. Scotting

  2. the Division of Child Health, Children's Brain Tumour Research Centre, School of Human Development, University of Nottingham, Queen's Medical Centre, Nottingham, NG7 2UH, UK

    David A. Walker

  3. the Division of Haematology-Oncology, Department of Paediatrics, University Hospital of Padua, via Giustiniani 3, 35128, Italy

    Giorgio Perilongo

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  1. Paul J. Scotting
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Correspondence to Paul J. Scotting.

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Related links

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DATABASES

Entrez Gene

α-fetoprotein

IGF2

p107

p53

PTCH

RB

SHH

TIMP1

WT1

WT2

National Cancer Institute

medulloblastoma

neuroblastoma

retinoblastoma

Wilms' tumour

OMIM

Gorlin's syndrome

Turcot's syndrome

Glossary

METANEPHRIC DEVELOPMENT

The metanephros is the permanent kidney of adult amniotes. This forms later than the transient pronephros of the early embryo.

NEURAL CREST

The neural crest is a multipotent population of cells that arises from the dorsal aspect of the early neural tube. These cells migrate widely throughout the body and form almost all of the components of the peripheral nervous system as well as other non-neural cell types such as the melanocytes of the skin and bone and muscle of the face.

SYMPATHOADRENAL

The sympathoadrenal lineage is one fate of neural crest cells and represents a common progenitor of the adrenergic sympathetic neurons and the medullary cells of the adrenal gland.

PURKINJE CELLS

Purkinje cells are very large neurons of the cerebellum with a highly arborized dendritic tree. They are responsible for all output from the cerebellar cortex.

URETERIC BUDS

Ureteric buds represent the leading edges of the epithelial tubules that grow and branch from the nephric ducts to form the kidney tubules. The ureteric buds signal to surrounding mesenchyme to form the nephrons.

NEPHROGENIC RESTS

Nephrogenic rests are clusters of developmentally immature cells and dysplastic cellular arrangements.

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Scotting, P., Walker, D. & Perilongo, G. Childhood solid tumours: a developmental disorder. Nat Rev Cancer 5, 481–488 (2005). https://doi.org/10.1038/nrc1633

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