Epidemiology of Glioma

Abstract

Giomas constitute a broad class of neuroectodermal tumours believed to originate from sustentacular neuroglial cells (Kleihues and Cavenee 2000). Astrocytomas form the largest group of gliomas (>75%) and glioblastoma multiforme (GBM) is the most common type of astrocytoma (CBTRUS 2011). Gliomas that share histologic characteristics with ependymal or oligodendrocyte cells are named ependymomas and oligodendrogliomas, but may not necessarily originate from the aforementioned cell types (Kleihues and Cavenee 2000). Mixed gliomas include those which consist of more than one glia cell type. For example, oligodendroglial glioblastoma multiforme (as defined by some neuropathologists) are GBM tumours with an oligodendroglioma component and generally have a significantly worse clinical outcome than GBM tumours overall (Louis et al 2007). Another mixed glioma is oligoastrocytoma, which contains both oligodendrocyte and astrocyte cells. The Third Edition of the International Classification of Diseases for oncology (ICD-O-3) is widely used to categorize gliomas by histology (e.g., malignant glioma=9380, ependymoma NOS=9391, astrocytoma=9430, glioblastoma NOS=9440, oligodendroglioma NOS=9450) (Fritz et al 2000). Furthermore, tumours are grouped by site in the ICD-O-3 system using Ccodes (e.g., cerebrum=C71.0, frontal lobal=C71.1, temporal lobe=C71.2, parietal lobe=C71.3, occipital lobe=C71.4, ventricle=C71.5, cerebellum=C71.6, spinal cord=C72.0). The World Health Organization (WHO) also has developed a classification index which grades gliomas by disease prognosis (I=best to IV=worst) (Kliehues et al 1993). Recent additions to the “WHO Classification of Tumours” include Grade I angiocentric gliomas (predominantly occurring in children and young adults in the fronto-parietal cortex, temporal lobe, and hippocampal region), and Grade II – pilomyxoid astrocytoma (typically occurring in infants and children in the hypothalamic/chiasmatic region) (Louis et al 2007). Additionally, WHO has recognized a divergent pattern of gliomas named small cell glioblastoma characterized by EGFR amplification, p16INK4a homozygous deletion, PTEN mutations, and LOH 10q (Louis et al 2007).