KEGG   DISEASE: GliomaHelp
H00042                      Disease                                

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.
Human diseases [BR:br08402]
  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]
BRITE hierarchy
hsa05214  Glioma
hsa05206  MicroRNAs in cancer
nt06273  Glioma
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
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]
X- and gamma-radiation
Carmustine [DR:D00254]
Temozolomide [DR:D06067]
Bevacizumab [DR:D06409]
Aminolevulinic acid hydrochloride [DR:D02908]
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
PMID:15639402 (gene, tumor type)
Soni D, King JA, Kaye AH, Hovens CM.
Genetics of glioblastoma multiforme: mitogenic signaling and cell cycle pathways converge.
J Clin Neurosci 12:1-5 (2005)
Holland EC.
Gliomagenesis: genetic alterations and mouse models.
Nat Rev Genet 2:120-9 (2001)
Gan HK, Cvrljevic AN, Johns TG
The epidermal growth factor receptor variant III (EGFRvIII): where wild things are altered.
FEBS J 280:5350-70 (2013)
Zhu Y, Parada LF.
The molecular and genetic basis of neurological tumours.
Nat Rev Cancer 2:616-26 (2002)
PMID:11604478 (carcinogen)
Bondy ML, Wang LE, El-Zein R, de Andrade M, Selvan MS, Bruner JM, Levin VA, Alfred Yung WK, Adatto P, Wei Q.
Gamma-radiation sensitivity and risk of glioma.
J Natl Cancer Inst 93:1553-7 (2001)
PMID:2790826 (carcinogen)
Preston-Martin S, Mack W, Henderson BE.
Risk factors for gliomas and meningiomas in males in Los Angeles County.
Cancer Res 49:6137-43 (1989)
PMID:12826827 (marker)
Hill C, Hunter SB, Brat DJ.
Genetic markers in glioblastoma: prognostic significance and future therapeutic implications.
Adv Anat Pathol 10:212-7 (2003)

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