{"entity":{"id":"oligodendroglioma","kind":"cancer","name":"Oligodendroglioma, IDH-mutant and 1p/19q-codeleted","aka":["Oligodendroglioma","Anaplastic oligodendroglioma","1p/19q codeleted glioma"],"tldr":"Oligodendroglioma is the adult brain tumour most responsive to chemotherapy. It is recognised by an IDH mutation together with loss of parts of chromosomes 1 and 19, and after surgery it is treated with radiotherapy plus the PCV drug combination, or, for small grade 2 tumours, with vorasidenib or watchful waiting.","summary":"Oligodendroglioma, IDH-mutant and 1p/19q-codeleted is defined in WHO 2021 by the combination of an IDH1 or IDH2 mutation with whole-arm codeletion of 1p and 19q; TERT promoter mutations are near universal and CIC and FUBP1 mutations common. It is graded 2 or 3 by mitotic activity, microvascular proliferation and necrosis; there is no grade 4. Calcification, frontal location and seizures are typical, and the tumour infiltrates cortex widely, so complete resection is often impossible.\n\nThe codeletion was first linked to chemosensitivity by Cairncross in 1998, and two trials that opened in the 1990s proved it: RTOG 9402 (radiotherapy with or without PCV before it) and EORTC 26951 (radiotherapy with or without PCV after it). In codeleted tumours the long-term reports in 2013 showed survival roughly doubled, with a median of 14.7 years against 7.3 years in RTOG 9402 and median survival not reached against 112 months in EORTC 26951, so radiotherapy followed by PCV became the standard for grade 3 disease and, after RTOG 9802, for high-risk grade 2 disease. CODEL is testing whether temozolomide can replace PCV, which is harder to tolerate; CATNON did not include codeleted tumours.\n\nFor grade 2 tumours with residual or recurrent disease and no pressing need for radiotherapy, INDIGO included oligodendroglioma alongside astrocytoma and vorasidenib is now an approved alternative to observation. Because patients live for many years, the cumulative cognitive effects of radiotherapy, the timing of treatment after surgery and the sequencing of vorasidenib, chemotherapy and radiotherapy are the live questions. Recurrent disease is treated with re-resection, temozolomide, lomustine or re-irradiation.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Oligodendroglioma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Oligodendroglioma"},{"label":"RTOG 9402 long-term results (JCO 2013)","url":"https://doi.org/10.1200/JCO.2012.43.2674"},{"label":"EORTC 26951 long-term results (JCO 2013)","url":"https://doi.org/10.1200/JCO.2012.43.2229"}],"tags":["subtype-page","cns"],"related":["glioblastoma","idh-mutant-astrocytoma","brain-tumours"],"cancers":[],"sections":[],"technologies":["methylation-profiling","imrt-igrt","extent-of-resection"],"targets":["idh"],"drugs":["procarbazine","lomustine","vincristine","vorasidenib","temozolomide"],"companies":[],"institutions":[],"pathways":["glioma-signalling"],"terms":["re-irradiation"],"trials":["rtog-9402","eortc-26951","indigo"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"central nervous system","burden":"A small share of adult gliomas, typically found in the frontal lobes of people in their thirties and forties; it has the best outlook of any diffuse adult glioma, with survival often measured in decades.","subtypes":["Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 2","Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 3 (formerly anaplastic)","Oligodendroglioma with minigemistocytes or gliofibrillary oligodendrocytes (morphological variants)"],"biomarkers":["IDH1 or IDH2 mutation","Whole-arm 1p/19q codeletion (FISH, array or methylation-based copy number)","TERT promoter mutation","CIC and FUBP1 mutations","CDKN2A/B homozygous deletion (poor outlook, rare)","DNA methylation class"],"standardOfCare":[{"setting":"Newly diagnosed","approach":"Maximal safe resection and molecular diagnosis with 1p/19q testing; observation is reasonable after gross total resection of a grade 2 tumour in a younger patient.","refs":["extent-of-resection","methylation-profiling","active-surveillance"],"guideline":{"version":"NCCN Guidelines: Central Nervous System Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1425"}},{"setting":"Grade 2, residual or recurrent, not needing radiotherapy","approach":"Vorasidenib (INDIGO) or continued observation.","refs":["vorasidenib","indigo"],"guideline":{"version":"NCCN Guidelines: Central Nervous System Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1425"}},{"setting":"Grade 2, high risk, and grade 3","approach":"Radiotherapy followed by PCV (procarbazine, lomustine, vincristine), the regimen validated in RTOG 9402, EORTC 26951 and RTOG 9802; radiotherapy with temozolomide is an alternative being compared in CODEL.","refs":["imrt-igrt","procarbazine","lomustine","vincristine","rtog-9402","eortc-26951","temozolomide"],"guideline":{"version":"NCCN Guidelines: Central Nervous System Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1425"}},{"setting":"Recurrence","approach":"Re-resection, temozolomide or lomustine, re-irradiation; bevacizumab for symptoms.","refs":["temozolomide","lomustine","re-irradiation","bevacizumab-glioma"]}],"stateOfArt":["Oligodendroglioma was the first brain tumour in which a chromosomal marker predicted benefit from chemotherapy, fifteen years before the trials confirmed it.","Radiotherapy plus PCV roughly doubled survival in codeleted tumours and is still the standard for grade 3 disease.","Vorasidenib gives grade 2 patients a drug option before radiotherapy for the first time."],"history":[{"year":1926,"title":"Bailey and Cushing describe oligodendroglioma as a distinct tumour","refs":[]},{"year":1998,"title":"Cairncross links 1p/19q loss to chemosensitivity","note":"Journal of the National Cancer Institute report that codeleted anaplastic oligodendrogliomas respond to PCV and live longer.","refs":["procarbazine","lomustine","vincristine"]},{"year":2006,"title":"RTOG 9402 and EORTC 26951 report: PCV lengthens progression-free but not yet overall survival","refs":["rtog-9402","eortc-26951"]},{"year":2013,"title":"Long-term results: radiotherapy plus PCV doubles survival in codeleted tumours","note":"RTOG 9402 median 14.7 versus 7.3 years; EORTC 26951 median not reached versus 112 months (both JCO).","refs":["rtog-9402","eortc-26951"]},{"year":2016,"title":"WHO 2016 defines oligodendroglioma by IDH mutation and 1p/19q codeletion","refs":[]},{"year":2016,"title":"RTOG 9802: radiotherapy plus PCV for high-risk grade 2 glioma, including oligodendroglioma","refs":["procarbazine","lomustine","vincristine"]},{"year":2023,"title":"INDIGO: vorasidenib in grade 2 astrocytoma and oligodendroglioma","refs":["vorasidenib","indigo"]},{"year":2024,"title":"FDA approves vorasidenib","refs":["vorasidenib"]}],"pipeline":["vorasidenib","safusidenib","methylation-profiling"],"openProblems":["Whether temozolomide can replace PCV without losing survival (CODEL).","How to sequence vorasidenib, chemotherapy and radiotherapy over a disease course of decades.","Late cognitive effects of radiotherapy in long survivors.","No effective treatment for the rare tumours that acquire CDKN2A/B loss or hypermutation."],"parent":"glioblastoma"},"route":"/cancers/oligodendroglioma/","neighbours":{"cancer":[{"id":"idh-mutant-astrocytoma","kind":"cancer","name":"Astrocytoma, IDH-mutant (grades 2 to 4)","route":"/cancers/idh-mutant-astrocytoma/"},{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","route":"/cancers/brain-tumours/"},{"id":"glioblastoma","kind":"cancer","name":"Glioma & glioblastoma","route":"/cancers/glioblastoma/"}],"technology":[{"id":"active-surveillance","kind":"technology","name":"Active surveillance","route":"/technologies/active-surveillance/"},{"id":"methylation-profiling","kind":"technology","name":"DNA methylation profiling","route":"/technologies/methylation-profiling/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"}],"term":[{"id":"extent-of-resection","kind":"term","name":"Extent of resection (RANO resect classes)","route":"/terms/extent-of-resection/"},{"id":"re-irradiation","kind":"term","name":"Re-irradiation","route":"/terms/re-irradiation/"}],"target":[{"id":"idh","kind":"target","name":"IDH1 / IDH2","route":"/targets/idh/"}],"drug":[{"id":"bevacizumab-glioma","kind":"drug","name":"Bevacizumab (glioblastoma use)","route":"/drugs/bevacizumab-glioma/"},{"id":"lomustine","kind":"drug","name":"Lomustine (CCNU)","route":"/drugs/lomustine/"},{"id":"procarbazine","kind":"drug","name":"Procarbazine","route":"/drugs/procarbazine/"},{"id":"safusidenib","kind":"drug","name":"Safusidenib","route":"/drugs/safusidenib/"},{"id":"temozolomide","kind":"drug","name":"Temozolomide","route":"/drugs/temozolomide/"},{"id":"vincristine","kind":"drug","name":"Vincristine","route":"/drugs/vincristine/"},{"id":"vorasidenib","kind":"drug","name":"Vorasidenib","route":"/drugs/vorasidenib/"}],"pathway":[{"id":"glioma-signalling","kind":"pathway","name":"Glioma (KEGG map)","route":"/pathways/glioma-signalling/"}],"trial":[{"id":"eortc-22033","kind":"trial","name":"EORTC 22033-26033","route":"/trials/eortc-22033/"},{"id":"eortc-26951","kind":"trial","name":"EORTC 26951","route":"/trials/eortc-26951/"},{"id":"indigo","kind":"trial","name":"INDIGO","route":"/trials/indigo/"},{"id":"rtog-9402","kind":"trial","name":"RTOG 9402","route":"/trials/rtog-9402/"}]}}