Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
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.
Overview
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.
The 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.
For 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.
State of the art
- 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.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Emergency services nowBowel perforation
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
- Emergency services nowDifferentiation syndrome
Fever, cough or breathlessness, rapid weight gain or swelling, bone pain, low blood pressure or reduced urine; the labels say to start steroids and monitor at the first suspicion, and the syndrome has been fatal.
- Check before combiningFood and drink: Temozolomide
Take on an empty stomach or at bedtime to reduce nausea; PJP prophylaxis during concurrent chemoradiation.
- Check before combiningFood and drink: Vincristine
Fatal if given intrathecally: label all syringes.
- Good to knowPeripheral neuropathy (chemotherapy-induced)
Nerve damage from chemotherapy that causes numbness, tingling and pain in the hands and feet, and sometimes weakness or hearing loss. It builds up with each dose, can be permanent, and is the main reason oxaliplatin, taxanes and vincristine have to be stopped or reduced.
See all on the product pages:Bevacizumab (glioblastoma use)Lomustine (CCNU)ProcarbazineTemozolomideVincristineVorasidenib·Printable cards in the navigator
Anatomy and lymph node drainage
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant glioma
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)
- Pineal and suprasellar midline (germ cell tumours)
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant gliomaOligodendroglioma, 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)
- Cerebellum (medulloblastoma)
- Brainstem and spinal cord (diffuse midline glioma, cord tumours)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)
- Deep periventricular tissue (CNS lymphoma)
- Meninges and convexity (meningioma)
- Grey-white junction (brain metastases)
- Cerebellopontine angle and eighth nerve (vestibular schwannoma)
- Pineal and suprasellar midline (germ cell tumours)
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural)
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- 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.
- DNA methylation profilingEstablished
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
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.
Vorasidenib (INDIGO) or continued observation.
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.
Re-resection, temozolomide or lomustine, re-irradiation; bevacizumab for symptoms.
Subtypes & biomarkers
top- 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)
- 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
How often this target appears
- 1926Bailey and Cushing describe oligodendroglioma as a distinct tumour
- 1998Cairncross links 1p/19q loss to chemosensitivity
Journal of the National Cancer Institute report that codeleted anaplastic oligodendrogliomas respond to PCV and live longer.
- 2006RTOG 9402 and EORTC 26951 report: PCV lengthens progression-free but not yet overall survival
- 2013Long-term results: radiotherapy plus PCV doubles survival in codeleted tumours
RTOG 9402 median 14.7 versus 7.3 years; EORTC 26951 median not reached versus 112 months (both JCO).
- 2016WHO 2016 defines oligodendroglioma by IDH mutation and 1p/19q codeletion
- 2016RTOG 9802: radiotherapy plus PCV for high-risk grade 2 glioma, including oligodendroglioma
- 2023INDIGO: vorasidenib in grade 2 astrocytoma and oligodendroglioma
- 2024FDA approves vorasidenib
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 15 changes by month →- 2026-09-17This recordOligodendroglioma, IDH-mutant and 1p/19q-codeletedFacts on this page last checked
When this page itself was last checked or edited.
- 2024-08-06RegulatoryVorasidenibVorasidenib: approval (US)
Grade 2 IDH-mutant astrocytoma or oligodendroglioma after surgery, age ≥12 (INDIGO): first targeted therapy for low-grade glioma
- 2024ApprovalVorasidenibVorasidenib approved in US
Grade 2 IDH-mutant astrocytoma or oligodendroglioma after surgery, age ≥12
- 2024MilestoneVorasidenibFDA approves vorasidenib
A milestone in how this cancer is treated.
- 2023Trial resultINDIGOINDIGO reported
PFS 27.
- 2023MilestoneVorasidenibINDIGO: vorasidenib in grade 2 astrocytoma and oligodendroglioma
A milestone in how this cancer is treated.
What is in development for Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, drawn from the whole corpus: 5 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Drugs in phase 3 · 1
Trials reported · 4
- EORTC 22033-26033 · phase 3 · 2016 · negative
- EORTC 26951 · phase 3 · 2013 · positive
- INDIGO · phase 3 · 2023 · positive
- RTOG 9402 · phase 3 · 2013 · positive
Open problems and what is being done
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.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Landmark trials
Expert centres
topExpert centres
Heidelberg · cancer center | Germany | none recorded | 0 | 3,456 | 45,745 | #18 | |
Brussels · consortium | Belgium | none recorded | 2 | not matched | - | none recorded | - |
Philadelphia, PA · consortium | United States | none recorded | 1 | 87 | 2,130 | none recorded | - |
Heidelberg · research institute | Germany | none recorded | 0 | 2,202 | 32,575 | - | |
Utrecht · cancer center | Netherlands | none recorded | 0 | 1,422 | 20,323 | - | |
Tianjin · cancer center | China | none recorded | 0 | 1,219 | 11,455 | - | |
Hangzhou · cancer center | China | none recorded | 0 | 1,219 | 17,635 | - | |
Rozzano (Milan) · hospital | Italy | none recorded | 0 | 1,031 | 10,720 | - | |
Changsha · cancer center | China | none recorded | 0 | 930 | 14,477 | - | |
Jinan · cancer center | China | none recorded | 0 | 920 | 7,804 | - | |
Dresden · cancer center | Germany | none recorded | 0 | 728 | 7,984 | - | |
| United Kingdom | none recorded | 0 | 693 | 7,168 | - | ||
Aarhus · hospital | Denmark | none recorded | 0 | 624 | 4,497 | - | |
Freiburg im Breisgau · cancer center | Germany | none recorded | 0 | 549 | 5,261 | - | |
Geneva · hospital | Switzerland | none recorded | 0 | 441 | 6,485 | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Oligodendroglioma, IDH-mutant and 1p/19q-codeleted but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example IDH1 or IDH2 mutation, Whole-arm 1p/19q codeletion, TERT promoter mutation, CIC and FUBP1 mutations, CDKN2A/B homozygous deletion), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 2, Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, grade 3, Oligodendroglioma with minigemistocytes or gliofibrillary oligodendrocytes.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
- For my situation (newly diagnosed), which of the standard options do you recommend and why?Why: Guideline options include: 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.
Grade 2, residual or recurrent, not needing radiotherapy
- For my situation (grade 2, residual or recurrent, not needing radiotherapy), which of the standard options do you recommend and why?Why: Guideline options include: Vorasidenib (INDIGO) or continued observation.
- Am I a candidate for Vorasidenib, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of INDIGO apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Grade 2, high risk, and grade 3
- For my situation (grade 2, high risk, and grade 3), which of the standard options do you recommend and why?Why: Guideline options include: 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.
- Am I a candidate for Procarbazine, Lomustine (CCNU), Vincristine or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of RTOG 9402 and EORTC 26951 apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Recurrence
- For my situation (recurrence), which of the standard options do you recommend and why?Why: Guideline options include: Re-resection, temozolomide or lomustine, re-irradiation; bevacizumab for symptoms.
- Am I a candidate for Temozolomide, Lomustine (CCNU), Bevacizumab (glioblastoma use), and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Vorasidenib, Safusidenib, DNA methylation profiling?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Whether temozolomide can replace PCV without losing survival (CODEL)”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “How to sequence vorasidenib, chemotherapy and radiotherapy over a disease course of decades”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
8targets
2drugs
7companies
4pathways
1terms
2trials
4Latest papers
topQuery for this cancer: (TITLE:"Oligodendroglioma, IDH-mutant and 1p/19q-codeleted" OR ABSTRACT:"Oligodendroglioma, IDH-mutant and 1p/19q-codeleted" OR TITLE:"Oligodendroglioma" OR ABSTRACT:"Oligodendroglioma" OR TITLE:"Anaplastic oligodendroglioma" OR ABSTRACT:"Anaplastic oligodendroglioma" OR TITLE:"1p/19q codeleted glioma" OR ABSTRACT:"1p/19q codeleted glioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, not a curated reading list.
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