Abstract
Significant advances in our understanding of the molecular genetics of pediatric and adult brain tumors and the resulting rapid expansion of clinical molecular neuropathology have led to improvements in diagnostic accuracy and identified new targets for therapy. Moreover, there have been major improvements in all facets of clinical care, including imaging, surgery, radiation and supportive care. In selected cohorts of patients, targeted and immunotherapies have resulted in improved patient outcomes. Furthermore, adaptations to clinical trial design have facilitated our study of new agents and other therapeutic innovations. However, considerable work remains to be done towards extending survival for all patients with primary brain tumors, especially children and adults with diffuse midline gliomas harboring Histone H3 K27 mutations and adults with isocitrate dehydrogenase (IDH) wild-type, O6 guanine DNA-methyltransferase gene (MGMT) promoter unmethylated high grade gliomas. In addition to improvements in therapy and care, access to the advances in technology, such as particle radiation or biologic therapy, neuroimaging and molecular diagnostics in both developing and developed countries is needed to improve the outcome of patients with brain tumors.
Highlights
If one could infer by attendance at major neuro-oncology conferences and the representation of pediatric and adult neuro-oncology at international oncology meetings, there has been an influx of new investigators, interest and significant advances in biomedical research pertaining to improving diagnosis, risk stratification, and treatment for children and adults with primary brain tumors
With the advent and subsequent implementation of platforms such as whole genome sequencing [2], single cell nucleic acid sequencing [3,4,5,6,7], nanostring technology [8, 9] and DNA methylation [10,11,12] profiling, some diagnostic categories have been replaced, such as the former primitive neuroepithelial tumor (PNET)grouping [13], whereas more common tumors such as low grade gliomas and glioblastoma (GBM) in pediatric and adult age groups are being split into subgroups specified by molecular and genetic considerations [14,15,16,17,18,19,20]
The Glioma Longitudinal Analysis Consortium (GLASS) was established to assess genomic, epigenomic and other molecular changes such as tumor mutational burden and mutational signatures that occur over time from initial diagnosis to tumor progression/recurrence, including in response to chemotherapy and radiation [19, 21]
Summary
If one could infer by attendance at major neuro-oncology conferences and the representation of pediatric and adult neuro-oncology at international oncology meetings, there has been an influx of new investigators, interest and significant advances in biomedical research pertaining to improving diagnosis, risk stratification, and treatment for children and adults with primary brain tumors. We discuss several topics of current interest to the neuro-oncology community to reflect the directions the field is taking
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