Abstract
Glioblastoma is an aggressive and inevitably recurrent primary intra-axial brain tumor with a dismal prognosis. The current mainstay of treatment involves maximally safe surgical resection followed by radiotherapy over a 6-week period with concomitant temozolomide chemotherapy followed by temozolomide maintenance. This review provides a summary of the epidemiological, clinical, histologic and genetic characteristics of newly diagnosed disease as well as the current standard of care and potential future therapeutic prospects.
Highlights
Glioblastoma, a World Health Organization grade IV astrocytoma, with an incidence in North America of 5.0 per 100,000 population, representing 15 to 20% of all primary intracranial neoplasms, in adults [1], is highly aggressive, with an unusually dismal prognosis [2], with a 5-year survival rate of 5%
The cIMPACT- Update 3 provided diagnostic criteria for “Diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma WHO Grade IV,” and recommended that histologic grade II and III IDH-wildtype diffuse astrocytic glioma that contain a high level of epidermal growth factor receptor (EGFR) amplification, the combination of whole chromosome 7 gain and whole chromosome 10 loss, or TERT promoter mutations, correspond to WHO grade IV and be referred to as diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma, WHO grade IV [23]
Glioblastoma, which accounts for about 15% of all central nervous system (CNS) cancers [41], is an orphan disease, with less than 20,000 cases in the United States
Summary
Glioblastoma, a World Health Organization grade IV astrocytoma, with an incidence in North America of 5.0 per 100,000 population, representing 15 to 20% of all primary intracranial neoplasms, in adults [1], is highly aggressive, with an unusually dismal prognosis (death typically results in the first 15-16 months after diagnosis) [2], with a 5-year survival rate of 5%. GBM recurrence is inevitable and virtually all patients will relapse, with the majority of relapses occurring centrally within 2 cm of the original gadolinium-enhanced mass on MRI [10] This accounts for its unfavorable prognosis, and, despite decades of investment and research, there is still an unmet medical need for a more efficacious treatment [11]. Quality of life in these patients is progressively and significantly impacted due to the emergence of debilitating symptoms arising from infiltrative tumor growth far into functionally intact brain tissue that restricts and disrupts normal day-to-day activities [13] For all these reasons, the identification and integration of new treatment options that enhance the cytotoxic effects of the standard-of-care radiation/temozolomide regimen constitute an urgent unmet medical need
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