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.
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.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
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.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Choroid plexus carcinoma, 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), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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Young adults with a grade 2 IDH-mutant glioma who have had surgery can now take a daily tablet that slows or reverses tumour growth and defers radiotherapy and chemotherapy, both of which carry long-term cognitive costs, by years. It confirms that the IDH mutation is a driver that can be targeted, not just a marker. Whether it improves survival or cognition over the long term, and whether it helps in higher-grade or previously treated tumours, is unknown.
Every brain tumour page on this site uses these names and grades; a tumour called glioblastoma before 2021 may now be an IDH-mutant astrocytoma with a different outlook and treatment.
Radiotherapy followed by PCV is the standard for high-risk grade 2 IDH-mutant glioma; whether temozolomide can replace PCV, and whether vorasidenib can defer both, are the current questions.
Together with RTOG 9402, this trial made radiotherapy followed by PCV the standard for 1p/19q-codeleted anaplastic oligodendroglioma; the CODEL trial is now comparing PCV with temozolomide.
1p/19q codeletion is a predictive marker: codeleted oligodendroglioma is treated with radiotherapy plus PCV, and the finding helped make the codeletion part of the tumour's definition.
Query 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.
Journal of the National Cancer Institute report that codeleted anaplastic oligodendrogliomas respond to PCV and live longer.
RTOG 9402 median 14.7 versus 7.3 years; EORTC 26951 median not reached versus 112 months (both JCO).
The targets of this cancer's medicines and the ones linked to it directly.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
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.
Take on an empty stomach or at bedtime to reduce nausea; PJP prophylaxis during concurrent chemoradiation.
Fatal if given intrathecally: label all syringes.
Alkylating chemotherapy can damage a blood stem cell in a way that shows up years later as myelodysplastic syndrome or acute myeloid leukaemia. It is uncommon, it depends on the total dose, and the risk falls away after about ten years. Knowing the cumulative dose you were given is the single most useful thing on your treatment summary.
See all on the product pages:Bevacizumab (glioblastoma use)Lomustine (CCNU)ProcarbazineTemozolomideVincristineVorasidenib·Printable cards in the navigator
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