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IDH-mutant astrocytoma is the slow-growing form of adult glioma, defined by a mutation in the IDH1 or IDH2 gene that makes the tumour produce a chemical which rewires its own cells. Surgery first, and then either watchful waiting, the new pill vorasidenib, or radiotherapy with chemotherapy, depending on grade and how much tumour is left.
Astrocytoma, IDH-mutant is a single WHO 2021 tumour type graded 2, 3 or 4, defined by an IDH1 or IDH2 mutation without 1p/19q codeletion and usually with ATRX loss and TP53 mutation. Grade 4 is assigned on necrosis, microvascular proliferation or homozygous CDKN2A/B deletion, and the old term glioblastoma is no longer used for IDH-mutant tumours. The mutant enzyme produces 2-hydroxyglutarate, which blocks demethylases and gives the tumour its hypermethylated (G-CIMP) phenotype; that dependence is the target of vorasidenib.
Maximal safe resection comes first, with awake mapping where language or motor cortex is close. For grade 2 disease with residual or recurrent tumour and no urgent need for radiotherapy, INDIGO (NEJM 2023) showed vorasidenib prolonged progression-free survival to a median of 27.7 months against 11.1 months on placebo and delayed the next intervention; the FDA approved it in August 2024 for grade 2 astrocytoma and oligodendroglioma from the age of 12. For higher-risk grade 2 disease (age 40 or over, or subtotal resection) RTOG 9802 showed radiotherapy followed by PCV chemotherapy lengthened median survival from 7.8 to 13.3 years compared with radiotherapy alone; EORTC 22033-26033 found temozolomide alone was not superior to radiotherapy alone. For grade 3 (1p/19q non-codeleted) tumours CATNON established radiotherapy followed by twelve cycles of adjuvant temozolomide; concurrent temozolomide added nothing overall. Grade 4 IDH-mutant tumours are treated with radiotherapy and temozolomide by extrapolation from glioblastoma.
At recurrence the options are re-resection, re-irradiation, lomustine or temozolomide re-challenge and bevacizumab for symptom control. Hypermutation after temozolomide, CDKN2A/B loss and methylation class shape prognosis. Open questions are whether vorasidenib should replace or merely defer radiotherapy, how to treat grade 3 and 4 IDH-mutant tumours with IDH inhibitors (safusidenib, olutasidenib and others are in trials), and how to weigh the cognitive cost of radiotherapy in people who will live with the disease for decades.
A minority of adult diffuse gliomas, presenting mostly in people in their twenties to forties, often with a seizure; it grows slowly for years and then transforms, so patients live with it for a long time and treatment is timed as much as chosen.
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), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, 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 with awake or functional mapping where needed; integrated histological and molecular diagnosis (IDH, 1p/19q, ATRX, CDKN2A/B, methylation class).
Observation with serial MRI, or vorasidenib for grade 2 tumours not needing immediate radiotherapy or chemotherapy (INDIGO).
Radiotherapy followed by PCV (procarbazine, lomustine, vincristine) as in RTOG 9802, or radiotherapy with temozolomide; temozolomide alone was not better than radiotherapy alone in EORTC 22033.
Radiotherapy followed by twelve cycles of adjuvant temozolomide (CATNON); concurrent temozolomide is not needed in non-codeleted tumours.
Radiotherapy with concurrent and adjuvant temozolomide, extrapolated from glioblastoma; trials of IDH inhibitors preferred where available.
Re-resection, re-irradiation, lomustine or temozolomide re-challenge, bevacizumab for oedema and symptoms; clinical trials.
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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 a year of temozolomide is the standard for grade 3 IDH-mutant astrocytoma; concurrent temozolomide is not needed, and IDH-wild-type tumours behave and are treated like glioblastoma.
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.
Query for this cancer: (TITLE:"Astrocytoma, IDH-mutant" OR ABSTRACT:"Astrocytoma, IDH-mutant" OR TITLE:"grades 2 to 4" OR ABSTRACT:"grades 2 to 4" OR TITLE:"IDH-mutant astrocytoma" OR ABSTRACT:"IDH-mutant astrocytoma" OR TITLE:"Diffuse astrocytoma, IDH-mutant" OR ABSTRACT:"Diffuse astrocytoma, IDH-mutant" OR TITLE:"Anaplastic astrocytoma, IDH-mutant" OR ABSTRACT:"Anaplastic astrocytoma, IDH-mutant" OR TITLE:"Lower-grade glioma" OR ABSTRACT:"Lower-grade glioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Astrocytoma, IDH-mutant (grades 2 to 4), not a curated reading list.
Parsons and colleagues sequence glioblastoma exomes (Science) and find IDH1 mutations concentrated in younger patients and secondary tumours.
Yan and colleagues (NEJM) show IDH mutations in most grade 2 and 3 gliomas and their better prognosis.
Buckner and colleagues (NEJM) report median overall survival of 13.3 years against 7.8 years with radiotherapy alone.
Mellinghoff and colleagues (NEJM): median progression-free survival 27.7 versus 11.1 months.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
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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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