Biological background of pediatric medulloblastoma and ependymoma: A review from a translational research perspective

Judith M. de Bont; Roger J. Packer; Erna M. Michiels; Monique L. den Boer; Rob Pieters

doi:10.1215/15228517-2008-059

Biological background of pediatric medulloblastoma and ependymoma: A review from a translational research perspective

Judith M. de Bont^*, Roger J. Packer, Erna M. Michiels, Monique L. den Boer, Rob Pieters

^*Corresponding author for this work

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

Abstract

Survival rates of pediatric brain tumor patients have significantly improved over the years due to developments in diagnostic techniques, neurosurgery, chemotherapy, radiotherapy, and supportive care. However, brain tumors are still an important cause of cancer-related deaths in children. Prognosis is still highly dependent on clinical characteristics, such as the age of the patient, tumor type, stage, and localization, but increased knowledge about the genetic and biological features of these tumors is being obtained and might be useful to further improve outcome for these patients. It has become clear that the deregulation of signaling pathways essential in brain development, for example, sonic hedgehog (SHH), Wnt, and Notch pathways, plays an important role in pathogenesis and biological behavior, especially for medulloblastomas. More recently, data have become available about the cells of origin of brain tumors and the possible existence of brain tumor stem cells. Newly developed array-based techniques for studying gene expression, protein expression, copy number aberrations, and epigenetic events have led to the identification of other potentially important biological abnormalities in pediatric medulloblastomas and ependymomas. Neuro-Oncology 10, 1040-1060, 2008 (Posted to Neuro-Oncology [serial online], Doc. D08-00067, August 1, 2008. URL http://neuro-oncology.dukejournals.org; DOI: 10.1215/15228517-2008-059)

Original language	English
Pages (from-to)	1040-1060
Number of pages	21
Journal	Neuro-Oncology
Volume	10
Issue number	6
DOIs	https://doi.org/10.1215/15228517-2008-059
Publication status	Published - Dec 2008
Externally published	Yes

Keywords

biological characteristics
ependymoma
epigenetic events
gene expression
medulloblastoma
protein expression
signaling pathways
COMPARATIVE GENOMIC HYBRIDIZATION
PRIMITIVE NEUROECTODERMAL TUMORS
CENTRAL-NERVOUS-SYSTEM
IN-SITU HYBRIDIZATION
MOLECULAR-GENETIC ANALYSIS
MESSENGER-RNA EXPRESSION
CPG ISLAND METHYLATION
HEDGEHOG-INDUCED MEDULLOBLASTOMA
ABERRANT PROMOTER METHYLATION
COPY NUMBER ABNORMALITIES

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10.1215/15228517-2008-059

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title = "Biological background of pediatric medulloblastoma and ependymoma: A review from a translational research perspective",

abstract = "Survival rates of pediatric brain tumor patients have significantly improved over the years due to developments in diagnostic techniques, neurosurgery, chemotherapy, radiotherapy, and supportive care. However, brain tumors are still an important cause of cancer-related deaths in children. Prognosis is still highly dependent on clinical characteristics, such as the age of the patient, tumor type, stage, and localization, but increased knowledge about the genetic and biological features of these tumors is being obtained and might be useful to further improve outcome for these patients. It has become clear that the deregulation of signaling pathways essential in brain development, for example, sonic hedgehog (SHH), Wnt, and Notch pathways, plays an important role in pathogenesis and biological behavior, especially for medulloblastomas. More recently, data have become available about the cells of origin of brain tumors and the possible existence of brain tumor stem cells. Newly developed array-based techniques for studying gene expression, protein expression, copy number aberrations, and epigenetic events have led to the identification of other potentially important biological abnormalities in pediatric medulloblastomas and ependymomas. Neuro-Oncology 10, 1040-1060, 2008 (Posted to Neuro-Oncology [serial online], Doc. D08-00067, August 1, 2008. URL http://neuro-oncology.dukejournals.org; DOI: 10.1215/15228517-2008-059)",

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author = "{de Bont}, {Judith M.} and Packer, {Roger J.} and Michiels, {Erna M.} and {den Boer}, {Monique L.} and Rob Pieters",

year = "2008",

month = dec,

doi = "10.1215/15228517-2008-059",

language = "English",

volume = "10",

pages = "1040--1060",

journal = "Neuro-Oncology",

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T1 - Biological background of pediatric medulloblastoma and ependymoma

T2 - A review from a translational research perspective

AU - de Bont, Judith M.

AU - Packer, Roger J.

AU - Michiels, Erna M.

AU - den Boer, Monique L.

AU - Pieters, Rob

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N2 - Survival rates of pediatric brain tumor patients have significantly improved over the years due to developments in diagnostic techniques, neurosurgery, chemotherapy, radiotherapy, and supportive care. However, brain tumors are still an important cause of cancer-related deaths in children. Prognosis is still highly dependent on clinical characteristics, such as the age of the patient, tumor type, stage, and localization, but increased knowledge about the genetic and biological features of these tumors is being obtained and might be useful to further improve outcome for these patients. It has become clear that the deregulation of signaling pathways essential in brain development, for example, sonic hedgehog (SHH), Wnt, and Notch pathways, plays an important role in pathogenesis and biological behavior, especially for medulloblastomas. More recently, data have become available about the cells of origin of brain tumors and the possible existence of brain tumor stem cells. Newly developed array-based techniques for studying gene expression, protein expression, copy number aberrations, and epigenetic events have led to the identification of other potentially important biological abnormalities in pediatric medulloblastomas and ependymomas. Neuro-Oncology 10, 1040-1060, 2008 (Posted to Neuro-Oncology [serial online], Doc. D08-00067, August 1, 2008. URL http://neuro-oncology.dukejournals.org; DOI: 10.1215/15228517-2008-059)

AB - Survival rates of pediatric brain tumor patients have significantly improved over the years due to developments in diagnostic techniques, neurosurgery, chemotherapy, radiotherapy, and supportive care. However, brain tumors are still an important cause of cancer-related deaths in children. Prognosis is still highly dependent on clinical characteristics, such as the age of the patient, tumor type, stage, and localization, but increased knowledge about the genetic and biological features of these tumors is being obtained and might be useful to further improve outcome for these patients. It has become clear that the deregulation of signaling pathways essential in brain development, for example, sonic hedgehog (SHH), Wnt, and Notch pathways, plays an important role in pathogenesis and biological behavior, especially for medulloblastomas. More recently, data have become available about the cells of origin of brain tumors and the possible existence of brain tumor stem cells. Newly developed array-based techniques for studying gene expression, protein expression, copy number aberrations, and epigenetic events have led to the identification of other potentially important biological abnormalities in pediatric medulloblastomas and ependymomas. Neuro-Oncology 10, 1040-1060, 2008 (Posted to Neuro-Oncology [serial online], Doc. D08-00067, August 1, 2008. URL http://neuro-oncology.dukejournals.org; DOI: 10.1215/15228517-2008-059)

KW - biological characteristics

KW - ependymoma

KW - epigenetic events

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KW - signaling pathways

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KW - PRIMITIVE NEUROECTODERMAL TUMORS

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KW - IN-SITU HYBRIDIZATION

KW - MOLECULAR-GENETIC ANALYSIS

KW - MESSENGER-RNA EXPRESSION

KW - CPG ISLAND METHYLATION

KW - HEDGEHOG-INDUCED MEDULLOBLASTOMA

KW - ABERRANT PROMOTER METHYLATION

KW - COPY NUMBER ABNORMALITIES

U2 - 10.1215/15228517-2008-059

DO - 10.1215/15228517-2008-059

M3 - Literature review

C2 - 18676356

SN - 1522-8517

VL - 10

SP - 1040

EP - 1060

JO - Neuro-Oncology

JF - Neuro-Oncology

IS - 6

ER -

Biological background of pediatric medulloblastoma and ependymoma: A review from a translational research perspective

Abstract

Keywords

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