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Glioma

Definition:

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.

References:

[1]. Deepa Soni, et al. Genetics of glioblastoma multiforme: mitogenic signaling and cell cycle pathways converge. J Clin Neurosci. 2005 Jan;12(1):1-5. [Content Brief]

[2]. E C Holland, et al. Gliomagenesis: genetic alterations and mouse models. Nat Rev Genet. 2001 Feb;2(2):120-9. [Content Brief]

[3]. F J T Staal, et al. A novel germline mutation of PTEN associated with brain tumours of multiple lineages. Br J Cancer. 2002 May 20;86(10):1586-91. [Content Brief]

[4]. Hongwu Zheng, et al. p53 and Pten control neural and glioma stem/progenitor cell renewal and differentiation. Nature. 2008 Oct 23;455(7216):1129-33. [Content Brief]

[5]. Hui K Gan, et al. The epidermal growth factor receptor variant III (EGFRvIII): where wild things are altered. FEBS J. 2013 Nov;280(21):5350-70. [Content Brief]

[6]. Jie Mei, et al. Clinical and molecular immune characterization of ERBB2 in glioma. Int Immunopharmacol. 2021 May;94:107499. [Content Brief]

[7]. Matthew N Bainbridge, et al. Germline mutations in shelterin complex genes are associated with familial glioma. J Natl Cancer Inst. 2014 Dec 7;107(1):384. [Content Brief]

[8]. S Reid, et al. Biallelic BRCA2 mutations are associated with multiple malignancies in childhood including familial Wilms tumour. J Med Genet. 2005 Feb;42(2):147-51. [Content Brief]

[9]. William A Flavahan, et al. Insulator dysfunction and oncogene activation in IDH mutant gliomas. Nature. 2016 Jan 7;529(7584):110-4. [Content Brief]

[10]. Yuan Zhu, et al. The molecular and genetic basis of neurological tumours. Nat Rev Cancer. 2002 Aug;2(8):616-26. [Content Brief]

Biomedical Dictionary

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