title: Glioma.
abstract: Gliomas are primary brain tumours that are thought to derive from neuroglial stem or progenitor cells. On the basis of their histological appearance, they have been traditionally classified as astrocytic, oligodendroglial or ependymal tumours and assigned WHO grades I-IV, which indicate different degrees of malignancy. Tremendous progress in genomic, transcriptomic and epigenetic profiling has resulted in new concepts of classifying and treating gliomas. Diffusely infiltrating gliomas in adults are now separated into three overarching tumour groups with distinct natural histories, responses to treatment and outcomes: isocitrate dehydrogenase (IDH)-mutant, 1p/19q co-deleted tumours with mostly oligodendroglial morphology that are associated with the best prognosis; IDH-mutant, 1p/19q non-co-deleted tumours with mostly astrocytic histology that are associated with intermediate outcome; and IDH wild-type, mostly higher WHO grade (III or IV) tumours that are associated with poor prognosis. Gliomas in children are molecularly distinct from those in adults, the majority being WHO grade I pilocytic astrocytomas characterized by circumscribed growth, favourable prognosis and frequent BRAF gene fusions or mutations. Ependymal tumours can be molecularly subdivided into distinct epigenetic subgroups according to location and prognosis. Although surgery, radiotherapy and alkylating agent chemotherapy are still the mainstay of treatment, individually tailored strategies based on tumour-intrinsic dominant signalling pathways and antigenic tumour profiles may ultimately improve outcome. For an illustrated summary of this Primer, visit: http://go.nature.com/TXY7Ri.
authors.affiliation: Department of Neurology and Brain Tumor Center, University Hospital Zurich and University of Zurich, Frauenklinikstrasse 26, CH-8091 Zurich, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Weller
authors.affiliation: Neurology Clinic, University of Heidelberg and German Cancer Research Center, Heidelberg, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Wick
authors.affiliation: Department of Pathology, University Health Network, Toronto, Ontario, Canada.
authors.forename: Ken
authors.initials: K
authors.lastname: Aldape
authors.affiliation: Department of Molecular and Clinical Cancer Medicine and Department of Radiation Oncology, University of Liverpool and Clatterbridge Cancer Centre NHS Foundation Trust, Liverpool, UK.
authors.forename: Michael
authors.initials: M
authors.lastname: Brada
authors.affiliation: Department of Neurological Surgery and Brain Tumor Research Center, University of California, San Francisco, California, USA.
authors.forename: Mitchell
authors.initials: M
authors.lastname: Berger
authors.affiliation: Division of Pediatric Neuro-Oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.|Department of Pediatric Haematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany.
authors.forename: Stefan M
authors.initials: SM
authors.lastname: Pfister
authors.affiliation: Department of Neuro-Oncology and Neurosurgery, Saitama Medical University, Saitama, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Nishikawa
authors.affiliation: Department of Medical Oncology, The Royal Melbourne Hospital, Victoria 3050, Australia.
authors.forename: Mark
authors.initials: M
authors.lastname: Rosenthal
authors.affiliation: Center for Neuro-Oncology, Dana-Farber/Brigham and Women's Cancer Center, Boston, Massachusetts, USA.
authors.forename: Patrick Y
authors.initials: PY
authors.lastname: Wen
authors.affiliation: Department of Oncology and Brain Tumor Center, University Hospital Zurich and University of Zurich, Zurich, Switzerland.
authors.forename: Roger
authors.initials: R
authors.lastname: Stupp
authors.affiliation: Department of Neuropathology, Heinrich Heine University Düsseldorf, and German Cancer Consortium (DKTK), German Cancer Research Center (DKFZ) Heidelberg, partner site Essen/Düsseldorf, Germany.
authors.forename: Guido
authors.initials: G
authors.lastname: Reifenberger
mesh_terms: D000328:Adult; D001932:Brain Neoplasms / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D002648:Child; D005910:Glioma / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D006801:Humans; D009154:Mutation; D060787:Neoplasm Grading / Q000379:methods; D011379:Prognosis