Journal of Neuro-Oncology

, Volume 70, Issue 2, pp 203-215

First online:

Molecular pathogenesis of childhood brain tumors

  • Torsten PietschAffiliated withDepartment of Neuropathology, University of Bonn Medical Center
  • , Michael D. TaylorAffiliated withDivision of Neurosurgery, Hospital for Sick Children
  • , James T. RutkaAffiliated withDivision of Neurosurgery, Hospital for Sick Children

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In the last decade, the molecular biology revolution has advanced considerably. These advances have enhanced our understanding of the genetic underpinnings of human brain tumors in general, and pediatric brain tumors in particular. We now know that many pediatric brain tumors arise from disturbances in developmentally regulated signaling pathways. The medulloblastoma, a tumor in which the developmental Hedgehog and WNT pathways have gone awry, is a prime example of this. New techniques in genetic engineering have allowed for the creation of sophisticated mouse models of brain tumors that recapitulate the human disease. Many laboratories are now using cDNA microarrays to study the expression level of thousands of genes that may be aberrantly expressed in brain tumors when compared to normal control cells. In the next decade, the use of several new molecular techniques to establish brain tumor diagnoses will likely become standard tools in the diagnostics and treatment stratification of children with central nervous system tumors.

atypical teratoid/rhabdoid tumor choroid plexus tumors ependymoma medulloblastoma molecular genetics pilocytic astrocytoma