{"entity":{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","aka":["Brain Tumors","Brain tumors","CNS tumours","Central nervous system tumours","Brain cancer"],"tldr":"Brain and spinal cord tumours range from slow-growing meningiomas and low-grade gliomas to glioblastoma, the commonest malignant brain tumour in adults, and a distinct set of childhood tumours such as medulloblastoma and diffuse midline glioma. Molecular markers now define them, and treatment is surgery, radiotherapy and, for some, drugs chosen by those markers.","summary":"Tumours of the brain and spinal cord are classified by the WHO by cell type and, since 2016 and more so in 2021, by molecular markers such as IDH mutation, 1p/19q codeletion, H3 K27 alteration and MGMT methylation. In adults the main groups are gliomas (from IDH-mutant low-grade astrocytomas and oligodendrogliomas to IDH-wildtype glioblastoma), meningiomas, pituitary tumours and primary CNS lymphoma; in children, medulloblastoma, ependymoma, low-grade gliomas, diffuse midline glioma and rare embryonal tumours dominate. Surgery as complete as function allows, radiotherapy and temozolomide remain the backbone; targeted drugs such as vorasidenib for IDH-mutant glioma and BRAF and MEK inhibitors for BRAF-altered paediatric glioma are the first molecular therapies to change practice. The blood-brain barrier and the impossibility of wide margins make these among the hardest cancers to treat, which is why the subtype pages give the detail.","asOf":"2026-09-04","wikipedia":"https://en.wikipedia.org/wiki/Brain_tumor","links":[{"label":"NCI: Brain tumors","url":"https://www.cancer.gov/types/brain"},{"label":"GLOBOCAN 2020 (Sung 2021)","url":"https://doi.org/10.3322/caac.21660"}],"tags":["umbrella"],"related":["glioblastoma","dipg-dmg","medulloblastoma","ependymoma","craniopharyngioma","atrt","paediatric-low-grade-glioma","primary-cns-lymphoma"],"cancers":[],"sections":[],"technologies":["bbb-focused-ultrasound"],"targets":["idh","braf","mek"],"drugs":["temozolomide","vorasidenib","dabrafenib","trametinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"notes":[],"group":"central nervous system","burden":"About 308,000 new cases and 251,000 deaths worldwide in 2020 (GLOBOCAN) for brain and central nervous system cancers; the leading cause of cancer death in children in many high-income countries.","subtypes":["Glioblastoma (IDH-wildtype)","IDH-mutant astrocytoma and oligodendroglioma","Paediatric low-grade glioma","Diffuse midline glioma (DIPG)","Medulloblastoma","Ependymoma","Atypical teratoid/rhabdoid tumour","Craniopharyngioma","Meningioma","Primary CNS lymphoma","Brain metastases (from other cancers)"],"biomarkers":["IDH1/IDH2 mutation","1p/19q codeletion","MGMT promoter methylation","H3 K27M alteration","BRAF V600E and KIAA1549::BRAF fusion in paediatric glioma","Molecular subgroups of medulloblastoma (WNT, SHH, group 3, group 4)"],"standardOfCare":[{"setting":"Glioblastoma","approach":"Maximal safe resection, radiotherapy with concurrent and adjuvant temozolomide (Stupp regimen), tumour-treating fields as an option. See the glioblastoma page.","refs":["temozolomide"]},{"setting":"IDH-mutant low-grade glioma","approach":"Surgery, then observation or vorasidenib for grade 2 tumours, radiotherapy and chemotherapy for higher risk.","refs":["vorasidenib"]},{"setting":"Childhood tumours","approach":"Risk-adapted surgery, radiotherapy and chemotherapy by subgroup; BRAF and MEK inhibitors for BRAF-altered low-grade glioma. See the medulloblastoma, ependymoma and DIPG pages.","refs":["dabrafenib","trametinib"]}],"stateOfArt":[],"history":[{"year":2005,"title":"Temozolomide chemoradiation becomes the glioblastoma standard","note":"The Stupp trial showed radiotherapy with concurrent and adjuvant temozolomide prolonged survival.","refs":["temozolomide"]},{"year":2016,"title":"WHO classification adds molecular markers","note":"IDH mutation, 1p/19q codeletion and H3 K27M began to define brain tumour types alongside microscopy; extended in 2021.","refs":[]},{"year":2024,"title":"Vorasidenib approved for IDH-mutant glioma","note":"First targeted drug for grade 2 astrocytoma and oligodendroglioma after surgery (INDIGO trial).","refs":["vorasidenib"]}],"pipeline":["dordaviprone","tovorafenib"],"openProblems":["Drugs rarely cross the blood-brain barrier at useful concentrations.","Diffuse midline glioma remains almost uniformly fatal.","Cognitive late effects of radiotherapy in children."]},"route":"/cancers/brain-tumours/","neighbours":{"cancer":[{"id":"atrt","kind":"cancer","name":"Atypical teratoid/rhabdoid tumour (ATRT)","route":"/cancers/atrt/"},{"id":"craniopharyngioma","kind":"cancer","name":"Craniopharyngioma","route":"/cancers/craniopharyngioma/"},{"id":"dipg-dmg","kind":"cancer","name":"Diffuse midline glioma, H3 K27-altered (including DIPG)","route":"/cancers/dipg-dmg/"},{"id":"ependymoma","kind":"cancer","name":"Ependymoma","route":"/cancers/ependymoma/"},{"id":"glioblastoma","kind":"cancer","name":"Glioma & glioblastoma","route":"/cancers/glioblastoma/"},{"id":"medulloblastoma","kind":"cancer","name":"Medulloblastoma","route":"/cancers/medulloblastoma/"},{"id":"paediatric-low-grade-glioma","kind":"cancer","name":"Paediatric low-grade glioma","route":"/cancers/paediatric-low-grade-glioma/"},{"id":"primary-cns-lymphoma","kind":"cancer","name":"Primary CNS lymphoma","route":"/cancers/primary-cns-lymphoma/"}],"technology":[{"id":"bbb-focused-ultrasound","kind":"technology","name":"Focused-ultrasound blood-brain barrier opening","route":"/technologies/bbb-focused-ultrasound/"}],"target":[{"id":"braf","kind":"target","name":"BRAF","route":"/targets/braf/"},{"id":"idh","kind":"target","name":"IDH1 / IDH2","route":"/targets/idh/"},{"id":"mek","kind":"target","name":"MEK1/2","route":"/targets/mek/"}],"drug":[{"id":"dabrafenib","kind":"drug","name":"Dabrafenib","route":"/drugs/dabrafenib/"},{"id":"dordaviprone","kind":"drug","name":"Dordaviprone","route":"/drugs/dordaviprone/"},{"id":"temozolomide","kind":"drug","name":"Temozolomide","route":"/drugs/temozolomide/"},{"id":"tovorafenib","kind":"drug","name":"Tovorafenib","route":"/drugs/tovorafenib/"},{"id":"trametinib","kind":"drug","name":"Trametinib","route":"/drugs/trametinib/"},{"id":"vorasidenib","kind":"drug","name":"Vorasidenib","route":"/drugs/vorasidenib/"}]}}