KEGG   DISEASE: GliomaHelp
Entry
H00042                      Disease                                

Name
Glioma
Description
Gliomas are the most common of the primary brain tumors and account for more than 40% of all central nervous system neoplasms. Gliomas include tumours that are composed predominantly of astrocytes (astrocytomas), oligodendrocytes (oligodendrogliomas), mixtures of various glial cells (for example,oligoastrocytomas) and ependymal cells (ependymomas). The most malignant form of infiltrating astrocytoma - glioblastoma multiforme (GBM) - is one of the most aggressive human cancers. GBM may develop de novo (primary glioblastoma) or by progression from low-grade or anaplastic astrocytoma (secondary glioblastoma). Primary glioblastomas develop in older patients and typically show genetic alterations (EGFR amplification, p16/INK4a deletion, and PTEN mutations) at frequencies of 24-34%. Secondary glioblastomas develop in younger patients and frequently show overexpression of PDGF and CDK4 as well as p53 mutations (65%) and loss of Rb playing major roles in such transformations. Loss of PTEN has been implicated in both pathways, although it is much more common in the pathogenesis of primary GBM.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of eye, brain, and central nervous system
   H00042  Glioma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of brain or central nervous system
   2A00  Primary neoplasms of brain
    H00042  Glioma
Cancer-accociated carbohydrates [br08441.html]
 H00042
BRITE hierarchy
Pathway
hsa05214  Glioma
hsa05206  MicroRNAs in cancer
Network
N00006  Amplified EGFR to RAS-ERK signaling pathway
N00016  PDGF-overexpression to RAS-ERK signaling pathway
N00018  Amplified PDGFR to RAS-ERK signaling pathway
N00027  Amplified EGFR to PLCG-CAMK signaling pathway
N00029  Amplified PDGFR to PLCG-CAMK signaling pathway
N00035  Amplified EGFR to PI3K signaling pathway
N00040  Amplified PDGFR to PI3K signaling pathway
N00041  EGFR-overexpression to RAS-ERK signaling pathway
N00042  EGFR-overexpression to PI3K signaling pathway
N00051  Deleted PTEN to PI3K signaling pathway
N00052  Mutation-inactivated PTEN to PI3K signaling pathway
N00067  Deleted p14(ARF) to p21-cell cycle G1/S
N00068  Amplified MDM2 to p21-cell cycle G1/S
N00071  Deleted p16(INK4a) to p16-cell cycle G1/S
N00072  Amplified CDK4 to cell cycle G1/S
N00074  Loss of RB1 to cell cycle G1/S
N00115  Mutation-inactivated TP53 to transcription
N00513  Mutation-activated EGFR to RAS-ERK signaling pathway
N00514  Mutation-activated EGFR to PI3K signaling pathway
 » show all
Gene
EGFR (amplification, mutation, overexpression) [HSA:1956] [KO:K04361]
MDM2 (amplification, overexpression) [HSA:4193] [KO:K06643]
PTEN (mutation) [HSA:5728] [KO:K01110]
p16/INK4A (deletion) [HSA:1029] [KO:K06621]
PDGF-A (overexpression) [HSA:5154] [KO:K04359]
PDGF-B (overexpression) [HSA:5155] [KO:K17386]
PDGFR-alpha (overexpression, amplification) [HSA:5156] [KO:K04363]
PDGFR-beta (overexpression, amplification) [HSA:5159] [KO:K05089]
CDK4 (amplification) [HSA:1019] [KO:K02089]
p53 (mutation) [HSA:7157] [KO:K04451]
RB1 (loss) [HSA:5925] [KO:K06618]
Carcinogen
X- and gamma-radiation
Drug
Carmustine [DR:D00254]
Temozolomide [DR:D06067]
Bevacizumab [DR:D06409]
Comment
ICD-O: 9401/3, Tumor type: Anaplastic astrocytoma
ICD-O: 9440/3, Tumor type: Glioblastoma
Other DBs
ICD-11: 2A00.0
ICD-10: C71
MeSH: D005910
Reference
PMID:15639402 (gene, tumor type)
  Authors
Soni D, King JA, Kaye AH, Hovens CM.
  Title
Genetics of glioblastoma multiforme: mitogenic signaling and cell cycle pathways converge.
  Journal
J Clin Neurosci 12:1-5 (2005)
DOI:10.1016/j.jocn.2004.04.001
Reference
  Authors
Holland EC.
  Title
Gliomagenesis: genetic alterations and mouse models.
  Journal
Nat Rev Genet 2:120-9 (2001)
DOI:10.1038/35052535
Reference
  Authors
Gan HK, Cvrljevic AN, Johns TG
  Title
The epidermal growth factor receptor variant III (EGFRvIII): where wild things are altered.
  Journal
FEBS J 280:5350-70 (2013)
DOI:10.1111/febs.12393
Reference
  Authors
Zhu Y, Parada LF.
  Title
The molecular and genetic basis of neurological tumours.
  Journal
Nat Rev Cancer 2:616-26 (2002)
DOI:10.1038/nrc866
Reference
PMID:11604478 (carcinogen)
  Authors
Bondy ML, Wang LE, El-Zein R, de Andrade M, Selvan MS, Bruner JM, Levin VA, Alfred Yung WK, Adatto P, Wei Q.
  Title
Gamma-radiation sensitivity and risk of glioma.
  Journal
J Natl Cancer Inst 93:1553-7 (2001)
DOI:10.1093/jnci/93.20.1553
Reference
PMID:2790826 (carcinogen)
  Authors
Preston-Martin S, Mack W, Henderson BE.
  Title
Risk factors for gliomas and meningiomas in males in Los Angeles County.
  Journal
Cancer Res 49:6137-43 (1989)
Reference
PMID:12826827 (marker)
  Authors
Hill C, Hunter SB, Brat DJ.
  Title
Genetic markers in glioblastoma: prognostic significance and future therapeutic implications.
  Journal
Adv Anat Pathol 10:212-7 (2003)
LinkDB All DBs

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