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0
Mappings
0
Definitions
0
Inheritance
5
Pathophysiology
1
Histopathology
3
Phenotypes
5
Pathograph
6
Genes
4
Treatments
2
Subtypes
0
Differentials
0
Datasets
0
Trials
0
Models
15
References
1
Deep Research
🏷

Classifications

Harrison's Chapter
cancer solid tumor
ICD-O Morphology
Glioma

Subtypes

2
Oligodendroglioma Grade 2
Low-grade oligodendroglioma with IDH mutation and 1p/19q codeletion. Characterized by uniform round nuclei, perinuclear halos (fried egg appearance), delicate branching capillaries (chicken-wire vasculature), and low mitotic activity. Median survival exceeds 15 years with appropriate treatment.
Oligodendroglioma Grade 3 (Anaplastic)
Anaplastic oligodendroglioma with IDH mutation and 1p/19q codeletion. Shows increased cellularity, mitotic activity, microvascular proliferation, and/or necrosis. Despite high-grade features, chemosensitivity is retained and median survival is 10-15 years with combined chemoradiation.

Pathophysiology

5
IDH1/2 Neomorphic Mutation
Heterozygous IDH1 R132 or IDH2 R172 mutations produce D-2-hydroxyglutarate (2-HG), an oncometabolite causing epigenetic dysregulation through inhibition of alpha-ketoglutarate-dependent dioxygenases. This is an early clonal event shared with IDH-mutant astrocytomas.
oligodendrocyte link
tricarboxylic acid cycle link ⚠ ABNORMAL
brain link
Show evidence (1 reference)
PMID:40916936 SUPPORT
"This unique immune microenvironment is shaped by 2-hydroxyglutarate (2-HG), an oncometabolite produced by mutant IDH."
This abstract explicitly links mutant IDH to 2-HG production, supporting the neomorphic IDH mechanism.
1p/19q Codeletion
The 1p/19q codeletion results from an unbalanced translocation t(1;19)(q10;p10) occurring early in tumorigenesis. This leads to loss of tumor suppressor genes including CIC on 19q and FUBP1 on 1p. The codeletion is associated with oligodendroglial differentiation and exceptional chemosensitivity.
chromosome organization link ⚠ ABNORMAL
Show evidence (1 reference)
PMID:37743332 SUPPORT
"Oligodendrogliomas were clearly defined as tumors with IDH mutations and 1p/19q codeletion by the World Health Organization(WHO)in 2016."
Abstract defines oligodendrogliomas by IDH mutation and 1p/19q codeletion.
G-CIMP Hypermethylation Phenotype
Glioma CpG island methylator phenotype (G-CIMP) results from 2-HG-mediated inhibition of TET enzymes and histone demethylases. This leads to widespread promoter hypermethylation, altered gene expression, and blocked differentiation. G-CIMP is associated with favorable prognosis.
DNA methylation link ↑ INCREASED
CIC/FUBP1 Tumor Suppressor Loss
CIC (Capicua) on 19q13 is mutated in approximately 70% of oligodendrogliomas. FUBP1 on 1p31 is mutated in approximately 30%. These mutations cooperate with IDH mutation and 1p/19q codeletion to drive oligodendroglioma formation.
negative regulation of cell population proliferation link ↓ DECREASED
Oligodendroglial Tumorigenesis
The combination of IDH mutation, 1p/19q codeletion, and CIC/FUBP1 mutations drives oligodendroglioma formation. These tumors retain oligodendroglial differentiation markers and maintain sensitivity to DNA-damaging agents, likely due to intact DNA damage response pathways.
oligodendrocyte link
cell population proliferation link ↑ INCREASED

Histopathology

1
Diffuse Glioma VERY_FREQUENT
Diffuse gliomas include IDH-mutant astrocytoma and oligodendroglioma subtypes.
Show evidence (1 reference)
PMID:36651583 SUPPORT
"oligodendroglioma IDH-mutant and 1p/19q codeleted, astrocytoma IDH-mutant, and"
Abstract lists IDH-mutant oligodendroglioma and astrocytoma among diffuse gliomas.

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for IDH-Mutant and 1p/19q-Codeleted Oligodendroglioma Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.

Phenotypes

3
Seizures VERY_FREQUENT Neurological HP:0001250
Show evidence (1 reference)
PMID:26478444 SUPPORT
"Epilepsy develops in more than 70-90% of oligodendroglial tumors and represents a favorable indicator for long-term survival if present as the first clinical sign."
Documents the high frequency (70-90%) of seizures in oligodendroglial tumors and their prognostic significance.
Headache FREQUENT Neurological HP:0002315
Cognitive Impairment FREQUENT Neurological HP:0100543
🧬

Genetic Associations

6
IDH1 (Somatic Mutation)
IDH2 (Somatic Mutation)
CIC (Somatic Mutation)
Show evidence (2 references)
PMID:22072542 SUPPORT
"overall mutation rate in oligodendrogliomas in this study to 20/29 (69%)"
Documents CIC mutation frequency of 69% in 1p/19q-codeleted oligodendrogliomas through exome sequencing.
PMID:22072542 SUPPORT
"CIC mutations were highly associated with oligodendroglioma histology, 1p/19q co-deletion, and IDH1/2 mutation (p < 0.001)"
Establishes strong association between CIC mutations and the defining features of oligodendroglioma.
FUBP1 (Somatic Mutation)
TERT (Promoter Mutation)
NOTCH1 (Somatic Mutation)
💊

Treatments

4
Maximal Safe Resection
Action: surgical procedure MAXO:0000004
Surgical resection aims to maximize extent of resection while preserving function. Complete resection associated with improved survival and seizure control.
Radiation Therapy
Action: radiation therapy MAXO:0000014
External beam radiation is standard for anaplastic oligodendroglioma. For grade 2 tumors, timing of radiation may be deferred in favorable cases.
PCV Chemotherapy
Action: chemotherapy MAXO:0000647
Procarbazine, CCNU (lomustine), and vincristine (PCV) combination chemotherapy shows exceptional efficacy in 1p/19q-codeleted oligodendrogliomas. RTOG 9402 and EORTC 26951 trials demonstrated major survival benefit for PCV added to radiation in anaplastic oligodendroglioma.
Show evidence (2 references)
PMID:23071247 SUPPORT
"the median survival of those with codeleted tumors treated with PCV plus RT was twice that of patients receiving RT (14.7 v 7.3 years; HR = 0.59; 95% CI, 0.37 to 0.95; P = .03)"
Landmark RTOG 9402 phase III trial demonstrating that PCV plus radiation doubles median survival compared to radiation alone in 1p/19q-codeleted anaplastic oligodendroglioma.
PMID:23071237 SUPPORT
"The addition of six cycles of PCV after 59.4 Gy of RT increases both OS and PFS in anaplastic oligodendroglial tumors. 1p/19q-codeleted tumors derive more benefit from adjuvant PCV compared with non-1p/19q-deleted tumors."
EORTC 26951 phase III trial confirming survival benefit of adjuvant PCV chemotherapy with preferential benefit in 1p/19q-codeleted tumors.
Temozolomide Chemotherapy
Action: chemotherapy MAXO:0000647
Temozolomide is an alternative to PCV with better tolerability. Often used concurrently with radiation followed by adjuvant cycles. Efficacy appears similar to PCV in 1p/19q-codeleted tumors.
🔬

Biochemical Markers

1
2-Hydroxyglutarate (2-HG)
{ }

Source YAML

click to show
name: IDH-Mutant and 1p/19q-Codeleted Oligodendroglioma
creation_date: '2026-01-26T02:55:13Z'
updated_date: '2026-04-11T21:17:25Z'
description: >-
  Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, is a diffuse glioma defined
  by
  the combination of IDH1/IDH2 mutation and whole-arm codeletion of chromosomes 1p
  and
  19q. This molecular signature is the result of an unbalanced translocation t(1;19)
  (q10;p10) and is associated with distinct biology, excellent chemosensitivity, and
  favorable prognosis. The 1p/19q codeletion is mutually exclusive with TP53 and ATRX
  mutations, serving as a key diagnostic discriminator from IDH-mutant astrocytoma.
  These tumors are exquisitely sensitive to alkylating chemotherapy, particularly
  procarbazine-CCNU-vincristine (PCV) combination, with long-term survival even for
  anaplastic tumors.
categories:
- Central Nervous System Neoplasm
- Adult Brain Tumor
- Molecularly Defined Tumor
- Chemosensitive Tumor
parents:
- diffuse glioma
has_subtypes:
- name: Oligodendroglioma Grade 2
  description: >-
    Low-grade oligodendroglioma with IDH mutation and 1p/19q codeletion. Characterized
    by uniform round nuclei, perinuclear halos (fried egg appearance), delicate
    branching capillaries (chicken-wire vasculature), and low mitotic activity.
    Median survival exceeds 15 years with appropriate treatment.
- name: Oligodendroglioma Grade 3 (Anaplastic)
  description: >-
    Anaplastic oligodendroglioma with IDH mutation and 1p/19q codeletion. Shows
    increased cellularity, mitotic activity, microvascular proliferation, and/or
    necrosis. Despite high-grade features, chemosensitivity is retained and
    median survival is 10-15 years with combined chemoradiation.
pathophysiology:
- name: IDH1/2 Neomorphic Mutation
  description: >-
    Heterozygous IDH1 R132 or IDH2 R172 mutations produce D-2-hydroxyglutarate (2-HG),
    an oncometabolite causing epigenetic dysregulation through inhibition of
    alpha-ketoglutarate-dependent dioxygenases. This is an early clonal event shared
    with IDH-mutant astrocytomas.
  evidence:
  - reference: PMID:40916936
    reference_title: "Isocitrate dehydrogenase mutation and microenvironment in gliomas: do immunotherapy approaches matter?"
    supports: SUPPORT
    snippet: "This unique immune microenvironment is shaped by 2-hydroxyglutarate (2-HG), an oncometabolite produced by mutant IDH."
    explanation: This abstract explicitly links mutant IDH to 2-HG production, supporting the neomorphic IDH mechanism.
  cell_types:
  - preferred_term: oligodendrocyte
    term:
      id: CL:0000128
      label: oligodendrocyte
  biological_processes:
  - preferred_term: tricarboxylic acid cycle
    modifier: ABNORMAL
    term:
      id: GO:0006099
      label: tricarboxylic acid cycle
  locations:
  - preferred_term: brain
    term:
      id: UBERON:0000955
      label: brain
  downstream:
  - target: G-CIMP Hypermethylation Phenotype
    description: 2-HG accumulation causes global DNA hypermethylation
- name: 1p/19q Codeletion
  description: >-
    The 1p/19q codeletion results from an unbalanced translocation t(1;19)(q10;p10)
    occurring early in tumorigenesis. This leads to loss of tumor suppressor genes
    including CIC on 19q and FUBP1 on 1p. The codeletion is associated with
    oligodendroglial differentiation and exceptional chemosensitivity.
  evidence:
  - reference: PMID:37743332
    reference_title: "[Oligodendroglioma, IDH Mutation and 1p/19q Codeletion]."
    supports: SUPPORT
    snippet: "Oligodendrogliomas were clearly defined as tumors with IDH mutations and 1p/19q codeletion by the World Health Organization(WHO)in 2016."
    explanation: "Abstract defines oligodendrogliomas by IDH mutation and 1p/19q codeletion."
  biological_processes:
  - preferred_term: chromosome organization
    modifier: ABNORMAL
    term:
      id: GO:0051276
      label: chromosome organization
  downstream:
  - target: CIC/FUBP1 Tumor Suppressor Loss
    description: Codeletion removes key tumor suppressors on 1p and 19q
- name: G-CIMP Hypermethylation Phenotype
  description: >-
    Glioma CpG island methylator phenotype (G-CIMP) results from 2-HG-mediated
    inhibition of TET enzymes and histone demethylases. This leads to widespread
    promoter hypermethylation, altered gene expression, and blocked differentiation.
    G-CIMP is associated with favorable prognosis.
  biological_processes:
  - preferred_term: DNA methylation
    modifier: INCREASED
    term:
      id: GO:0006304
      label: DNA modification
- name: CIC/FUBP1 Tumor Suppressor Loss
  description: >-
    CIC (Capicua) on 19q13 is mutated in approximately 70% of oligodendrogliomas.
    FUBP1 on 1p31 is mutated in approximately 30%. These mutations cooperate with
    IDH mutation and 1p/19q codeletion to drive oligodendroglioma formation.
  biological_processes:
  - preferred_term: negative regulation of cell population proliferation
    modifier: DECREASED
    term:
      id: GO:0008285
      label: negative regulation of cell population proliferation
  downstream:
  - target: Oligodendroglial Tumorigenesis
    description: Loss of tumor suppressors promotes oligodendroglioma formation
- name: Oligodendroglial Tumorigenesis
  description: >-
    The combination of IDH mutation, 1p/19q codeletion, and CIC/FUBP1 mutations
    drives oligodendroglioma formation. These tumors retain oligodendroglial
    differentiation markers and maintain sensitivity to DNA-damaging agents,
    likely due to intact DNA damage response pathways.
  cell_types:
  - preferred_term: oligodendrocyte
    term:
      id: CL:0000128
      label: oligodendrocyte
  biological_processes:
  - preferred_term: cell population proliferation
    modifier: INCREASED
    term:
      id: GO:0008283
      label: cell population proliferation
phenotypes:
- category: Neurological
  name: Seizures
  frequency: VERY_FREQUENT
  description: >-
    Seizures are the most common presenting symptom, occurring in 70-90% of patients.
    The high seizure frequency is attributed to cortical involvement and slow growth
    pattern. Seizure control often improves with tumor treatment.
  phenotype_term:
    preferred_term: Seizure
    term:
      id: HP:0001250
      label: Seizure
  evidence:
  - reference: PMID:26478444
    reference_title: "Seizures in oligodendroglial tumors."
    supports: SUPPORT
    snippet: "Epilepsy develops in more than 70-90% of oligodendroglial tumors and represents a favorable indicator for long-term survival if present as the first clinical sign."
    explanation: Documents the high frequency (70-90%) of seizures in oligodendroglial tumors and their prognostic significance.
- category: Neurological
  name: Headache
  frequency: FREQUENT
  description: >-
    Headache from mass effect occurs less commonly than in higher-grade tumors due
    to slow growth.
  phenotype_term:
    preferred_term: Headache
    term:
      id: HP:0002315
      label: Headache
- category: Neurological
  name: Cognitive Impairment
  frequency: FREQUENT
  description: >-
    Cognitive dysfunction may be subtle and develop slowly. Frontal lobe tumors may
    cause personality changes and executive dysfunction.
  phenotype_term:
    preferred_term: Cognitive impairment
    term:
      id: HP:0100543
      label: Cognitive impairment
histopathology:
- name: Diffuse Glioma
  finding_term:
    preferred_term: Diffuse Glioma
    term:
      id: NCIT:C129325
      label: Diffuse Glioma
  frequency: VERY_FREQUENT
  description: Diffuse gliomas include IDH-mutant astrocytoma and oligodendroglioma subtypes.
  evidence:
  - reference: PMID:36651583
    reference_title: "IDH-mutant diffuse gliomas: tips and tricks in the era of genomic tumor classification."
    supports: SUPPORT
    snippet: "oligodendroglioma IDH-mutant and 1p/19q codeleted, astrocytoma IDH-mutant, and"
    explanation: Abstract lists IDH-mutant oligodendroglioma and astrocytoma among diffuse gliomas.

genetic:
- name: IDH1
  association: Somatic Mutation
  notes: >-
    IDH1 R132H is the most common mutation but IDH1 R132C and other variants occur
    more frequently in oligodendrogliomas than astrocytomas.
- name: IDH2
  association: Somatic Mutation
  notes: >-
    IDH2 R172 mutations are more common in oligodendrogliomas (up to 5%) than
    astrocytomas.
- name: CIC
  association: Somatic Mutation
  notes: >-
    CIC (Capicua transcriptional repressor) on 19q13.2 is mutated in approximately
    70% of oligodendrogliomas. Functions as a transcriptional repressor downstream
    of receptor tyrosine kinase signaling.
  evidence:
  - reference: PMID:22072542
    reference_title: "Concurrent CIC mutations, IDH mutations, and 1p/19q loss distinguish oligodendrogliomas from other cancers."
    supports: SUPPORT
    snippet: "overall mutation rate in oligodendrogliomas in this study to 20/29 (69%)"
    explanation: Documents CIC mutation frequency of 69% in 1p/19q-codeleted oligodendrogliomas through exome sequencing.
  - reference: PMID:22072542
    reference_title: "Concurrent CIC mutations, IDH mutations, and 1p/19q loss distinguish oligodendrogliomas from other cancers."
    supports: SUPPORT
    snippet: "CIC mutations were highly associated with oligodendroglioma histology, 1p/19q co-deletion, and IDH1/2 mutation (p < 0.001)"
    explanation: Establishes strong association between CIC mutations and the defining features of oligodendroglioma.
- name: FUBP1
  association: Somatic Mutation
  notes: >-
    FUBP1 (far upstream element binding protein 1) on 1p31.1 is mutated in
    approximately 30% of oligodendrogliomas. Regulates MYC expression.
- name: TERT
  association: Promoter Mutation
  notes: >-
    TERT promoter mutations (C228T or C250T) occur in approximately 70-80% of
    oligodendrogliomas. Creates ETS binding sites leading to TERT upregulation
    and telomerase activation.
- name: NOTCH1
  association: Somatic Mutation
  notes: >-
    NOTCH1 mutations occur in approximately 10-15% of oligodendrogliomas and may
    be associated with worse prognosis.
biochemical:
- name: 2-Hydroxyglutarate (2-HG)
  notes: >-
    D-2-hydroxyglutarate accumulates due to IDH mutation. Detectable by MR
    spectroscopy at 2.25 ppm and serves as a biomarker for response assessment.
treatments:
- name: Maximal Safe Resection
  description: >-
    Surgical resection aims to maximize extent of resection while preserving
    function. Complete resection associated with improved survival and seizure
    control.
  treatment_term:
    preferred_term: surgical procedure
    term:
      id: MAXO:0000004
      label: surgical procedure
- name: Radiation Therapy
  description: >-
    External beam radiation is standard for anaplastic oligodendroglioma. For
    grade 2 tumors, timing of radiation may be deferred in favorable cases.
  treatment_term:
    preferred_term: radiation therapy
    term:
      id: MAXO:0000014
      label: radiation therapy
- name: PCV Chemotherapy
  description: >-
    Procarbazine, CCNU (lomustine), and vincristine (PCV) combination chemotherapy
    shows exceptional efficacy in 1p/19q-codeleted oligodendrogliomas. RTOG 9402
    and EORTC 26951 trials demonstrated major survival benefit for PCV added to
    radiation in anaplastic oligodendroglioma.
  treatment_term:
    preferred_term: chemotherapy
    term:
      id: MAXO:0000647
      label: chemotherapy
  evidence:
  - reference: PMID:23071247
    reference_title: "Phase III trial of chemoradiotherapy for anaplastic oligodendroglioma: long-term results of RTOG 9402."
    supports: SUPPORT
    snippet: "the median survival of those with codeleted tumors treated with PCV plus RT was twice that of patients receiving RT (14.7 v 7.3 years; HR = 0.59; 95% CI, 0.37 to 0.95; P = .03)"
    explanation: Landmark RTOG 9402 phase III trial demonstrating that PCV plus radiation doubles median survival compared to radiation alone in 1p/19q-codeleted anaplastic oligodendroglioma.
  - reference: PMID:23071237
    reference_title: "Adjuvant procarbazine, lomustine, and vincristine chemotherapy in newly diagnosed anaplastic oligodendroglioma: long-term follow-up of EORTC brain tumor group study 26951."
    supports: SUPPORT
    snippet: "The addition of six cycles of PCV after 59.4 Gy of RT increases both OS and PFS in anaplastic oligodendroglial tumors. 1p/19q-codeleted tumors derive more benefit from adjuvant PCV compared with non-1p/19q-deleted tumors."
    explanation: EORTC 26951 phase III trial confirming survival benefit of adjuvant PCV chemotherapy with preferential benefit in 1p/19q-codeleted tumors.
- name: Temozolomide Chemotherapy
  description: >-
    Temozolomide is an alternative to PCV with better tolerability. Often used
    concurrently with radiation followed by adjuvant cycles. Efficacy appears
    similar to PCV in 1p/19q-codeleted tumors.
  treatment_term:
    preferred_term: chemotherapy
    term:
      id: MAXO:0000647
      label: chemotherapy
disease_term:
  preferred_term: IDH-mutant and 1p/19q-codeleted oligodendroglioma
  term:
    id: MONDO:0859592
    label: IDH-mutant and 1p/19q-codeleted oligodendroglioma

classifications:
  icdo_morphology:
    classification_value: Glioma
  harrisons_chapter:
  - classification_value: cancer
  - classification_value: solid tumor
references:
- reference: DOI:10.1002/1878-0261.13598
  title: '<scp>IDH</scp> mutation, glioma immunogenicity, and therapeutic challenge of primary mismatch repair deficient <scp>IDH</scp>‐mutant astrocytoma <scp>PMMRDIA</scp>: a systematic review'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas.
    supporting_text: In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas.
    evidence:
    - reference: DOI:10.1002/1878-0261.13598
      reference_title: '<scp>IDH</scp> mutation, glioma immunogenicity, and therapeutic challenge of primary mismatch repair deficient <scp>IDH</scp>‐mutant astrocytoma <scp>PMMRDIA</scp>: a systematic review'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1007/s10014-022-00446-1
  title: 'Update of the 2021 WHO classification of tumors of the central nervous system: adult diffuse gliomas'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: 'Update of the 2021 WHO classification of tumors of the central nervous system: adult diffuse gliomas'
    supporting_text: 'Update of the 2021 WHO classification of tumors of the central nervous system: adult diffuse gliomas'
- reference: DOI:10.1007/s11060-023-04250-5
  title: Updates on the WHO diagnosis of IDH-mutant glioma
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: The WHO classification of Tumors of the Central Nervous System represents the international standard classification for brain tumors.
    supporting_text: The WHO classification of Tumors of the Central Nervous System represents the international standard classification for brain tumors.
    evidence:
    - reference: DOI:10.1007/s11060-023-04250-5
      reference_title: Updates on the WHO diagnosis of IDH-mutant glioma
      supports: SUPPORT
      evidence_source: OTHER
      snippet: The WHO classification of Tumors of the Central Nervous System represents the international standard classification for brain tumors.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1038/s41582-022-00679-w
  title: Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours
    supporting_text: Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours
- reference: DOI:10.1056/nejmoa2304194
  title: Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma
    supporting_text: Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma
- reference: DOI:10.1093/neuonc/noaa022
  title: Imaging growth as a predictor of grade of malignancy and aggressiveness of IDH-mutant and 1p/19q-codeleted oligodendrogliomas in adults
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: We quantified the spontaneous imaging growth rate of oligodendrogliomas.
    supporting_text: We quantified the spontaneous imaging growth rate of oligodendrogliomas.
    evidence:
    - reference: DOI:10.1093/neuonc/noaa022
      reference_title: Imaging growth as a predictor of grade of malignancy and aggressiveness of IDH-mutant and 1p/19q-codeleted oligodendrogliomas in adults
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: We quantified the spontaneous imaging growth rate of oligodendrogliomas.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1093/neuonc/noaa168
  title: 'CODEL: phase III study of RT, RT + TMZ, or TMZ for newly diagnosed 1p/19q codeleted oligodendroglioma. Analysis from the initial study design'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: We report the analysis involving patients treated on the initial CODEL design.
    supporting_text: We report the analysis involving patients treated on the initial CODEL design.
    evidence:
    - reference: DOI:10.1093/neuonc/noaa168
      reference_title: 'CODEL: phase III study of RT, RT + TMZ, or TMZ for newly diagnosed 1p/19q codeleted oligodendroglioma. Analysis from the initial study design'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: We report the analysis involving patients treated on the initial CODEL design.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1093/neuonc/noab106
  title: 'The 2021 WHO Classification of Tumors of the Central Nervous System: a summary'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
    supporting_text: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
    evidence:
    - reference: DOI:10.1093/neuonc/noab106
      reference_title: 'The 2021 WHO Classification of Tumors of the Central Nervous System: a summary'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1093/noajnl/vdaa109
  title: Frequency of false-positive FISH 1p/19q codeletion in adult diffuse astrocytic gliomas
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Oligodendroglioma is genetically defined by concomitant IDH (IDH1/IDH2) mutation and whole-arm 1p/19q codeletion.
    supporting_text: Oligodendroglioma is genetically defined by concomitant IDH (IDH1/IDH2) mutation and whole-arm 1p/19q codeletion.
    evidence:
    - reference: DOI:10.1093/noajnl/vdaa109
      reference_title: Frequency of false-positive FISH 1p/19q codeletion in adult diffuse astrocytic gliomas
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Oligodendroglioma is genetically defined by concomitant IDH (IDH1/IDH2) mutation and whole-arm 1p/19q codeletion.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1186/s13073-023-01175-6
  title: Stalled oligodendrocyte differentiation in IDH-mutant gliomas
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Roughly 50% of adult gliomas harborisocitrate dehydrogenase(IDH) mutations.
    supporting_text: Roughly 50% of adult gliomas harborisocitrate dehydrogenase(IDH) mutations.
    evidence:
    - reference: DOI:10.1186/s13073-023-01175-6
      reference_title: Stalled oligodendrocyte differentiation in IDH-mutant gliomas
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Roughly 50% of adult gliomas harborisocitrate dehydrogenase(IDH) mutations.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.1200/jco.21.02543
  title: 'Joint Final Report of EORTC 26951 and RTOG 9402: Phase III Trials With Procarbazine, Lomustine, and Vincristine Chemotherapy for Anaplastic Oligodendroglial Tumors'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Clinical trials frequently include multiple end points that mature at different times.
    supporting_text: Clinical trials frequently include multiple end points that mature at different times.
    evidence:
    - reference: DOI:10.1200/jco.21.02543
      reference_title: 'Joint Final Report of EORTC 26951 and RTOG 9402: Phase III Trials With Procarbazine, Lomustine, and Vincristine Chemotherapy for Anaplastic Oligodendroglial Tumors'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Clinical trials frequently include multiple end points that mature at different times.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.32074/1591-951x-823
  title: Adult type diffuse gliomas in the new 2021 WHO Classification
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Adult type diffuse gliomas in the new 2021 WHO Classification
    supporting_text: Adult type diffuse gliomas in the new 2021 WHO Classification
- reference: DOI:10.3390/biomedicines12061349
  title: 'The 2021 World Health Organization Central Nervous System Tumor Classification: The Spectrum of Diffuse Gliomas'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types.
    supporting_text: The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types.
    evidence:
    - reference: DOI:10.3390/biomedicines12061349
      reference_title: 'The 2021 World Health Organization Central Nervous System Tumor Classification: The Spectrum of Diffuse Gliomas'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.3390/brainsci13050817
  title: 'From Theory to Practice: Implementing the WHO 2021 Classification of Adult Diffuse Gliomas in Neuropathology Diagnosis'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: Diffuse gliomas are the most common type of primary central nervous system (CNS) neoplasm to affect the adult population.
    supporting_text: Diffuse gliomas are the most common type of primary central nervous system (CNS) neoplasm to affect the adult population.
    evidence:
    - reference: DOI:10.3390/brainsci13050817
      reference_title: 'From Theory to Practice: Implementing the WHO 2021 Classification of Adult Diffuse Gliomas in Neuropathology Diagnosis'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Diffuse gliomas are the most common type of primary central nervous system (CNS) neoplasm to affect the adult population.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
- reference: DOI:10.3390/cancers16152752
  title: 'Targeting Isocitrate Dehydrogenase (IDH) in Solid Tumors: Current Evidence and Future Perspectives'
  found_in:
  - IDH_Mutant_Oligodendroglioma-deep-research-falcon.md
  findings:
  - statement: The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response.
    supporting_text: The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response.
    evidence:
    - reference: DOI:10.3390/cancers16152752
      reference_title: 'Targeting Isocitrate Dehydrogenase (IDH) in Solid Tumors: Current Evidence and Future Perspectives'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response.
      explanation: Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
📚

References & Deep Research

References

15
<scp>IDH</scp> mutation, glioma immunogenicity, and therapeutic challenge of primary mismatch repair deficient <scp>IDH</scp>‐mutant astrocytoma <scp>PMMRDIA</scp>: a systematic review
1 finding
In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas.
"In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas."
Show evidence (1 reference)
"In 2021, Suwala et al. described Primary Mismatch Repair Deficient IDH‐mutant Astrocytoma (PMMRDIA) as a distinct group of gliomas."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Update of the 2021 WHO classification of tumors of the central nervous system: adult diffuse gliomas
1 finding
Update of the 2021 WHO classification of tumors of the central nervous system: adult diffuse gliomas
"Update of the 2021 WHO classification of tumors of the central nervous system: adult diffuse gliomas"
Updates on the WHO diagnosis of IDH-mutant glioma
1 finding
The WHO classification of Tumors of the Central Nervous System represents the international standard classification for brain tumors.
"The WHO classification of Tumors of the Central Nervous System represents the international standard classification for brain tumors."
Show evidence (1 reference)
"The WHO classification of Tumors of the Central Nervous System represents the international standard classification for brain tumors."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours
1 finding
Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours
"Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours"
Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma
1 finding
Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma
"Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma"
Imaging growth as a predictor of grade of malignancy and aggressiveness of IDH-mutant and 1p/19q-codeleted oligodendrogliomas in adults
1 finding
We quantified the spontaneous imaging growth rate of oligodendrogliomas.
"We quantified the spontaneous imaging growth rate of oligodendrogliomas."
Show evidence (1 reference)
DOI:10.1093/neuonc/noaa022 SUPPORT Human Clinical
"We quantified the spontaneous imaging growth rate of oligodendrogliomas."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
CODEL: phase III study of RT, RT + TMZ, or TMZ for newly diagnosed 1p/19q codeleted oligodendroglioma. Analysis from the initial study design
1 finding
We report the analysis involving patients treated on the initial CODEL design.
"We report the analysis involving patients treated on the initial CODEL design."
Show evidence (1 reference)
DOI:10.1093/neuonc/noaa168 SUPPORT Human Clinical
"We report the analysis involving patients treated on the initial CODEL design."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
The 2021 WHO Classification of Tumors of the Central Nervous System: a summary
1 finding
The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors.
"The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors."
Show evidence (1 reference)
"The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Frequency of false-positive FISH 1p/19q codeletion in adult diffuse astrocytic gliomas
1 finding
Oligodendroglioma is genetically defined by concomitant IDH (IDH1/IDH2) mutation and whole-arm 1p/19q codeletion.
"Oligodendroglioma is genetically defined by concomitant IDH (IDH1/IDH2) mutation and whole-arm 1p/19q codeletion."
Show evidence (1 reference)
DOI:10.1093/noajnl/vdaa109 SUPPORT Human Clinical
"Oligodendroglioma is genetically defined by concomitant IDH (IDH1/IDH2) mutation and whole-arm 1p/19q codeletion."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Stalled oligodendrocyte differentiation in IDH-mutant gliomas
1 finding
Roughly 50% of adult gliomas harborisocitrate dehydrogenase(IDH) mutations.
"Roughly 50% of adult gliomas harborisocitrate dehydrogenase(IDH) mutations."
Show evidence (1 reference)
"Roughly 50% of adult gliomas harborisocitrate dehydrogenase(IDH) mutations."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Joint Final Report of EORTC 26951 and RTOG 9402: Phase III Trials With Procarbazine, Lomustine, and Vincristine Chemotherapy for Anaplastic Oligodendroglial Tumors
1 finding
Clinical trials frequently include multiple end points that mature at different times.
"Clinical trials frequently include multiple end points that mature at different times."
Show evidence (1 reference)
DOI:10.1200/jco.21.02543 SUPPORT Human Clinical
"Clinical trials frequently include multiple end points that mature at different times."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Adult type diffuse gliomas in the new 2021 WHO Classification
1 finding
Adult type diffuse gliomas in the new 2021 WHO Classification
"Adult type diffuse gliomas in the new 2021 WHO Classification"
The 2021 World Health Organization Central Nervous System Tumor Classification: The Spectrum of Diffuse Gliomas
1 finding
The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types.
"The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types."
Show evidence (1 reference)
DOI:10.3390/biomedicines12061349 SUPPORT Human Clinical
"The 2021 edition of the World Health Organization (WHO) classification of central nervous system tumors introduces significant revisions across various tumor types."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
From Theory to Practice: Implementing the WHO 2021 Classification of Adult Diffuse Gliomas in Neuropathology Diagnosis
1 finding
Diffuse gliomas are the most common type of primary central nervous system (CNS) neoplasm to affect the adult population.
"Diffuse gliomas are the most common type of primary central nervous system (CNS) neoplasm to affect the adult population."
Show evidence (1 reference)
DOI:10.3390/brainsci13050817 SUPPORT Human Clinical
"Diffuse gliomas are the most common type of primary central nervous system (CNS) neoplasm to affect the adult population."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.
Targeting Isocitrate Dehydrogenase (IDH) in Solid Tumors: Current Evidence and Future Perspectives
1 finding
The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response.
"The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response."
Show evidence (1 reference)
DOI:10.3390/cancers16152752 SUPPORT Human Clinical
"The isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) enzymes are involved in key metabolic processes in human cells, regulating differentiation, proliferation, and oxidative damage response."
Deep research cited this publication as relevant literature for IDH Mutant Oligodendroglioma.

Deep Research

1
Falcon
1. Disease Information
Edison Scientific Literature 46 citations 2026-04-05T17:41:43.774618

1. Disease Information

1.1 Concise overview

In the 2021 WHO Classification of Tumours of the Central Nervous System (WHO CNS5), oligodendroglioma is defined as an adult-type diffuse glioma that is molecularly defined by an IDH1/IDH2 mutation together with whole-arm 1p/19q codeletion, and is graded as CNS WHO grade 2 or grade 3 within the tumor type (louis2021the2021who pages 8-9, louis2021the2021who pages 6-8, komori2023updateofthe pages 1-2).

1.2 Key identifiers and controlled vocabularies

  • WHO entity name: Oligodendroglioma, IDH-mutant and 1p/19q-codeleted (louis2021the2021who pages 8-9).
  • WHO grade range: CNS WHO grade 2 or 3 (louis2021the2021who pages 8-9, louis2021the2021who pages 9-10).
  • Ontology / database identifiers: Not comprehensively retrievable from the available sources in this run (e.g., ICD-10/ICD-11, MeSH, Orphanet, OMIM, disease-specific MONDO for the exact entity).

1.3 Synonyms and alternative names

  • Historical terminology: “oligodendroglioma” and “anaplastic oligodendroglioma” roughly correspond to WHO grade 2 and grade 3 tumors, respectively, but WHO CNS5 emphasizes molecularly defined integrated diagnoses (louis2021the2021who pages 8-9, roux2020imaginggrowthas pages 7-7).
  • Legacy mixed-histology term “oligoastrocytoma” is deprecated in modern practice because most such cases resolve into astrocytoma vs oligodendroglioma on molecular testing (horbinski2022clinicalimplicationsof pages 1-2).

1.4 Evidence source type

The classification statements summarized here are aggregated, disease-level resources (WHO CNS5 summaries and implementation reviews), supplemented by clinical trials and cohort studies (horbinski2022clinicalimplicationsof pages 1-2, louis2021the2021who pages 8-9).


2. Etiology

2.1 Disease causal factors (genetic/mechanistic)

  • Founder event: IDH1/IDH2 gain-of-function (neomorphic) mutation leading to 2-hydroxyglutarate (2-HG) accumulation and epigenetic remodeling (DNA/histone hypermethylation) (martin2023fromtheoryto pages 1-2, carosi2024targetingisocitratedehydrogenase pages 3-4).
  • Definitional chromosomal event: Whole-arm 1p/19q codeletion in the setting of IDH mutation defines oligodendroglioma in WHO CNS5 (martin2023fromtheoryto pages 2-4, louis2021the2021who pages 6-8).

2.2 Risk factors

  • Genetic/molecular trajectory: In WHO CNS5-aligned reviews, IDH-mutant diffuse gliomas follow two broad molecular trajectories: (i) IDH + TP53/ATRX (astrocytic), vs (ii) IDH + whole-arm 1p/19q codeletion + TERT promoter mutation (oligodendroglial; generally more favorable) (reuss2023updatesonthe pages 1-2).
  • Epidemiologic modifiers: Age is a key demographic correlate (typical adult onset; see Epidemiology), but robust environmental risk factors were not retrievable in the evidence assembled for this run.

2.3 Protective factors / gene–environment interactions

No high-quality, disease-specific protective factors or gene–environment interaction evidence was retrieved in the assembled corpus for this run.


3. Phenotypes (Clinical Presentation)

3.1 Typical symptom complex

Clinical presentation is often insidious and related to lesion location and intracranial pressure (antonelli2022adulttypediffuse pages 4-6). Across neuroradiology-focused WHO CNS5 reviews: - Seizures are frequently the initial symptom, plausibly due to cortical involvement (gue2024the2021world pages 7-9). - Other common symptoms include headache and cognitive/personality changes (notably with frontal lobe involvement) (gue2024the2021world pages 7-9).

3.2 Phenotype characteristics (onset, progression, frequency)

  • Age of onset: commonly adult (often third to fifth decades) (gue2024the2021world pages 7-9).
  • Progression: infiltrative growth is typical; malignant progression/grade transformation can occur over time (see Temporal Development) (roux2020imaginggrowthas pages 7-7).

3.3 Quality of life impact

High-quality, phenotype-specific QoL effect size estimates for this exact entity were not retrieved in this run. However, the long expected survival in many patients makes late treatment toxicity and “quality of survival” a major concern in treatment strategy discussions (carosi2024targetingisocitratedehydrogenase pages 4-6).

3.4 Suggested HPO terms (examples)

  • Seizures: HP:0001250
  • Headache: HP:0002315
  • Cognitive impairment: HP:0100543
  • Personality change: HP:0000751

4. Genetic/Molecular Information

4.1 Causal/definitional alterations

WHO CNS5 defines oligodendroglioma as requiring: - IDH1 or IDH2 mutation, and - Whole-arm 1p/19q codeletion (martin2023fromtheoryto pages 2-4, louis2021the2021who pages 6-8).

4.2 Common additional (characteristic) alterations

WHO-aligned molecular summaries list recurrent alterations in oligodendroglioma including TERT promoter, CIC, FUBP1, and NOTCH1 (martin2023fromtheoryto pages 4-6, louis2021the2021who pages 6-8).

4.3 Variant types and testing implications

  • IDH mutations are typically missense hotspot variants (e.g., IDH1 R132H is common; non-canonical IDH variants may require sequencing when IHC is negative but suspicion remains) (martin2023fromtheoryto pages 2-4, martin2023fromtheoryto pages 1-2).
  • Whole-arm 1p/19q codeletion is a structural/copy-number alteration, classically arising from an unbalanced translocation (conceptually consistent with modern diagnostic descriptions) and must be interpreted as whole-arm rather than partial loss (ball2020frequencyoffalsepositive pages 1-2).

4.4 Epigenetic information

  • IDH neomorphic activity and 2-HG accumulation are linked to DNA and histone hypermethylation and the glioma CpG island methylator phenotype (G‑CIMP) (martin2023fromtheoryto pages 1-2, carosi2024targetingisocitratedehydrogenase pages 3-4).

5. Environmental Information

No robust disease-specific environmental, lifestyle, or infectious causal factors were retrieved in the evidence assembled for this run.


6. Mechanism / Pathophysiology

6.1 Core causal chain (current understanding)

  1. IDH1/2 neomorphic mutation produces the oncometabolite 2-HG (martin2023fromtheoryto pages 1-2, carosi2024targetingisocitratedehydrogenase pages 3-4).
  2. 2-HG competitively inhibits α-KG–dependent dioxygenases (e.g., TET/Jumonji demethylases), causing DNA/histone hypermethylation and an epigenetically constrained cellular state (carosi2024targetingisocitratedehydrogenase pages 3-4).
  3. In IDH-mutant gliomas, this is linked to blocked differentiation programs and maintenance of an OPC-like developmental state (wei2023stalledoligodendrocytedifferentiation pages 1-2).

6.2 Differentiation blockade and cell-of-origin programs (2023 evidence)

A 2023 Genome Medicine multi-omic analysis concludes that IDH-mutant gliomas resemble early oligodendrocyte lineage states and show a blocked myelination program, supported by DNA methylation and chromatin accessibility patterns (wei2023stalledoligodendrocytedifferentiation pages 1-2).

6.3 Immune microenvironment effects (2024 evidence)

  • Reviews describe a mechanistic axis in which IDH-mutant tumors exhibit reduced immunogenicity and immune suppression linked to 2-HG and downstream epigenetic state; one review explicitly notes that “high levels of D-2-HG in the interstitial fluid of tumor cells” can impair T-cell proliferation and cytotoxicity (carosi2024targetingisocitratedehydrogenase pages 3-4).
  • A 2024 systematic review emphasizes immune suppression in IDH-mutant gliomas and highlights downstream consequences relevant to checkpoint blockade response (ahmad2024idhmutationglioma pages 1-2, ahmad2024idhmutationglioma pages 7-8).

6.4 Suggested ontology terms

  • GO biological process (examples): DNA methylation, histone methylation, glial cell differentiation, oligodendrocyte differentiation, T cell proliferation.
  • CL cell types (examples): oligodendrocyte progenitor cell (OPC), oligodendrocyte, microglial cell, T cell.

7. Anatomical Structures Affected

7.1 Organ/system level

  • Primary organ: brain (central nervous system).
  • Predilection for supratentorial cerebral hemispheres, especially frontal lobe (~60% reported in one WHO-aligned review), followed by temporal/parietal; occipital is less common; midline/posterior fossa/spinal are rare (antonelli2022adulttypediffuse pages 4-6, martin2023fromtheoryto pages 4-6).

7.2 Tissue/cell level

  • Tumor is a diffusely infiltrating glioma involving cortex and subcortical white matter (gue2024the2021world pages 7-9, martin2023fromtheoryto pages 4-6).

7.3 Suggested UBERON terms (examples)

  • Cerebral cortex; frontal lobe; cerebral white matter.

8. Temporal Development (Natural History)

8.1 Onset pattern

Often insidious presentation in adults (gue2024the2021world pages 7-9).

8.2 Progression and grading

  • WHO CNS5 recognizes grading within the entity: CNS WHO grade 2 and grade 3 oligodendroglioma (louis2021the2021who pages 8-9, louis2021the2021who pages 9-10).
  • Traditional grade 3 (“anaplastic”) criteria in earlier WHO frameworks incorporate brisk mitotic activity and/or microvascular proliferation; one study cites a cutoff of ≥6 mitoses per 10 high-power fields and highlights that imaging growth rate may capture aggressiveness and predict progression-free survival (roux2020imaginggrowthas pages 7-7).

9. Inheritance and Population

9.1 Inheritance

This tumor entity is primarily considered sporadic in routine clinical neuro-oncology practice; heritable Mendelian patterns were not supported by retrieved evidence in this run.

9.2 Epidemiology and demographics

A WHO CNS5 implementation review reports: - Incidence: approximately 0.48 per 100,000. - Age: peak in fourth–fifth decades. - Median overall survival: approximately 10–17 years (reflecting grade and treatment heterogeneity) (martin2023fromtheoryto pages 4-6).


10. Diagnostics

10.1 Integrated diagnostic criteria (WHO CNS5-aligned workflow)

A practical diagnostic workflow for adult diffuse gliomas is: 1. Test for IDH1/2 (IHC for IDH1 R132H and/or sequencing). 2. In IDH-mutant tumors, assess ATRX: loss supports astrocytoma; retained ATRX prompts 1p/19q testing. 3. If whole-arm 1p/19q codeletion is present, diagnose oligodendroglioma, IDH-mutant and 1p/19q-codeleted (martin2023fromtheoryto pages 2-4).

10.2 Key molecular tests and platforms

  • IDH: IHC (IDH1 R132H) and/or sequencing (martin2023fromtheoryto pages 2-4).
  • 1p/19q: FISH, chromosomal microarray (CMA), methylation array–derived copy number, or NGS-based copy-number approaches (martin2023fromtheoryto pages 4-6).

10.3 Important diagnostic caveat: false-positive FISH for 1p/19q

Because FISH is locus-based, it may not distinguish partial from whole-arm losses, producing “false-positive” 1p/19q results when partial losses mimic codeletion (ball2020frequencyoffalsepositive pages 1-2). In an adult diffuse astrocytic glioma series, the estimated false-positive FISH rate was 3.6% (8/223), with similar rates in IDH-mutant vs IDH-wildtype tumors (ball2020frequencyoffalsepositive pages 2-2). The authors recommend selective testing and/or confirmation with whole-arm–resolving platforms such as CMA when morphology/molecular context is discordant (ball2020frequencyoffalsepositive pages 2-2, ball2020frequencyoffalsepositive pages 13-14).

10.4 Imaging features used in real-world workflows (radiology)

Common imaging features reported across WHO-2021 radiology reviews include: - Location: frontal lobe predilection; cortical/subcortical involvement (gue2024the2021world pages 7-9, antonelli2022adulttypediffuse pages 4-6). - CT: hypodense/isodense lesion; calcifications are common (reported ~90% in one review) (martin2023fromtheoryto pages 4-6). - MRI: typically T1 hypointense, T2 hyperintense, often heterogeneous with indistinct margins (gue2024the2021world pages 9-12). - Contrast enhancement: variable; one review reports enhancement in <20% of grade 2 but >70% of grade 3 oligodendrogliomas (gue2024the2021world pages 9-12). - Perfusion: may show elevated rCBV reflecting vascularity (antonelli2022adulttypediffuse pages 4-6).


11. Outcome / Prognosis

11.1 Prognosis and survival statistics from pivotal trials (anaplastic/grade 3; codeleted)

Long-term randomized trial evidence (RTOG 9402 and EORTC 26951) demonstrates substantial benefit from adding PCV chemotherapy to radiotherapy in 1p/19q-codeleted anaplastic oligodendroglial tumors, with median OS on the order of a decade or longer and durable long-term survivors.

Key statistics include: - RTOG 9402 (JCO 2013): in codeleted tumors, median OS 14.7 years with PCV+RT vs 7.3 years with RT alone (HR 0.59; P=0.03) (cairncross2013phaseiiitrial pages 1-2). - Joint final report (JCO 2022): in the codeleted subgroup, probable 20-year OS ~37% with PCV+RT versus ~15% without PCV in RTOG 9402; and ~37% with PCV versus ~14% without PCV in EORTC 26951 (lassman2022jointfinalreport pages 1-2). The Kaplan–Meier curves and numbers-at-risk are shown in Figure 2 (lassman2022jointfinalreport media 697c589d).

11.2 Prognostic biomarkers (selected)

  • 1p/19q codeletion is strongly associated with improved outcomes relative to non-codeleted gliomas in the major randomized trial datasets (bent2013adjuvantprocarbazinelomustine pages 2-3, cairncross2013phaseiiitrial pages 1-2).
  • Epigenetic classifiers and additional CNV/alterations can stratify prognosis in IDH-mutant gliomas generally, but oligodendroglioma-specific prognostic molecular modeling was not deeply retrievable in this run.

12. Treatment

12.1 Standard-of-care components and real-world implementations

  • Maximal safe surgical resection followed by risk-adapted adjuvant therapy and MRI surveillance is a common backbone approach in guideline-aligned care pathways (carosi2024targetingisocitratedehydrogenase pages 4-6).
  • For grade 3 / anaplastic, randomized data support radiotherapy + PCV chemotherapy as a standard option in 1p/19q-codeleted tumors (lassman2022jointfinalreport pages 1-2, cairncross2013phaseiiitrial pages 1-2).

12.2 Temozolomide vs RT-containing regimens (evolving practice)

The initial CODEL trial design analysis showed inferior PFS with temozolomide alone compared with RT-containing regimens in newly diagnosed 1p/19q-codeleted grade 3 oligodendroglioma (jaeckle2021codelphaseiii pages 1-2). This led to redesign of CODEL to compare RT+PCV vs RT+TMZ rather than including a TMZ-only arm (jaeckle2021codelphaseiii pages 1-2, jaeckle2021codelphaseiii pages 9-10).

12.3 Targeted therapy (major 2023 development): IDH inhibitor vorasidenib

A major recent advance is the phase 3 INDIGO trial of vorasidenib in post-surgical residual/recurrent grade 2 IDH-mutant glioma (including oligodendroglioma and astrocytoma, stratified by 1p/19q status) (mellinghoff2023vorasidenibinidh1 pages 3-5).

Direct abstract quote supporting the key efficacy claim: - “Progression-free survival was significantly improved in the vorasidenib group as compared with the placebo group (median progression-free survival, 27.7 months vs. 11.1 months; hazard ratio … 0.39 …; P<0.001).” (mellinghoff2023vorasidenibinidh1 pages 1-3)

Safety signal of note: - Grade ≥3 ALT elevation occurred in 9.6% on vorasidenib vs 0% on placebo (mellinghoff2023vorasidenibinidh1 pages 16-18).

12.4 Suggested MAXO terms (examples)

  • Surgical tumor resection; external beam radiotherapy; chemotherapy with procarbazine/lomustine/vincristine; chemotherapy with temozolomide; targeted therapy with IDH inhibitor.

13. Prevention

Primary prevention and population-level screening strategies are not established for sporadic adult diffuse gliomas in the retrieved evidence. Secondary prevention largely consists of surveillance imaging in diagnosed patients following surgery and/or adjuvant therapy (carosi2024targetingisocitratedehydrogenase pages 4-6).


14. Other Species / Natural Disease

No naturally occurring non-human disease analogs were retrieved in this run.


15. Model Organisms

No specific oligodendroglioma model-organism systems were retrieved in the assembled evidence for this run; however, mechanistic multi-omics studies in human tumor samples (bulk and single-cell transcriptome, methylation, scATAC-seq) provide strong in situ evidence for differentiation blockade and epigenetic mechanisms (wei2023stalledoligodendrocytedifferentiation pages 1-2).


Key Recent Developments (2023–2024 emphasis)

  1. IDH inhibition in earlier-stage disease: INDIGO (NEJM 2023) demonstrated significant delay in progression and next intervention with vorasidenib in grade 2 IDH-mutant glioma after surgery only (mellinghoff2023vorasidenibinidh1 pages 1-3, mellinghoff2023vorasidenibinidh1 pages 3-5).
  2. Differentiation blockade mapped with multi-omics: 2023 single-cell/bulk multi-omics indicates stalled oligodendrocyte-lineage differentiation with blocked myelination programs in IDH-mutant gliomas (wei2023stalledoligodendrocytedifferentiation pages 1-2).
  3. Refinement of WHO CNS5 implementation: Practical guidance emphasizes integrated diagnoses driven by canonical molecular alterations and highlights laboratory workflow for IDH→ATRX→1p/19q testing (martin2023fromtheoryto pages 2-4, komori2023updateofthe pages 1-2).

Key Trials and Outcome Statistics (Table)

Trial Population Interventions Key efficacy outcomes Publication PMID URL
Joint Final Report: EORTC 26951 + RTOG 9402 Newly diagnosed anaplastic oligodendroglial tumors; key molecular subgroup: 1p/19q-codeleted tumors RT alone vs RT + PCV EORTC 26951, codeleted subgroup (n=80): median OS 9.3 y without PCV vs 14.2 y with PCV; HR 0.60 (95% CI 0.35-1.03), P=.063; 14-y OS 26.2% vs 51.0%; probable 20-y OS 13.6% vs 37.1%. RTOG 9402, codeleted subgroup (n=125): median OS 7.3 y without PCV vs 13.2 y with PCV; HR 0.61 (95% CI 0.40-0.94), P=.02; 14-y OS 25.0% vs 46.1%; probable 20-y OS 14.9% vs 37.0%. Median follow-up 18-19 y. (lassman2022jointfinalreport pages 1-2, lassman2022jointfinalreport pages 2-3) Journal of Clinical Oncology (2022) Not available in retrieved context https://doi.org/10.1200/JCO.21.02543
RTOG 9402 long-term results 291 eligible patients with anaplastic oligodendroglioma/oligoastrocytoma; 1p/19q-codeleted subgroup analyzed Intensive PCV then RT vs RT alone Overall cohort: median OS 4.6 y vs 4.7 y; HR 0.79 (95% CI 0.60-1.04), P=.1. Codeleted tumors: median OS 14.7 y with PCV+RT vs 7.3 y with RT alone; HR 0.59 (95% CI 0.37-0.95), P=.03. Non-codeleted: no benefit (2.6 y vs 2.7 y; HR 0.85, P=.39). (cairncross2013phaseiiitrial pages 1-2) Journal of Clinical Oncology (2013) Not available in retrieved context https://doi.org/10.1200/JCO.2012.43.2674
EORTC 26951 long-term follow-up 368 patients with newly diagnosed anaplastic oligodendroglioma; molecular data available for 316; key subgroup: 1p/19q-codeleted tumors RT alone vs RT followed by adjuvant PCV Overall cohort: median OS 30.6 mo with RT vs 42.3 mo with RT/PCV; HR 0.75 (95% CI 0.60-0.95). Median PFS 13.2 mo vs 24.3 mo; HR 0.66 (95% CI 0.52-0.83). Codeleted tumors: median PFS 76 mo vs 11 mo for non-codeleted; HR 0.39 (95% CI 0.28-0.53); median OS 123 mo vs 23 mo for non-codeleted; HR 0.36 (95% CI 0.27-0.49). In the codeleted treatment comparison, OS was not reached with RT/PCV vs 112 mo with RT; HR 0.56 (95% CI 0.31-1.03). (bent2013adjuvantprocarbazinelomustine pages 2-3, bent2013adjuvantprocarbazinelomustine pages 1-2) Journal of Clinical Oncology (2013) Not available in retrieved context https://doi.org/10.1200/JCO.2012.43.2229
CODEL initial design analysis Newly diagnosed 1p/19q-codeleted WHO grade 3 oligodendroglioma; 36 randomized patients RT alone vs RT + concomitant/adjuvant TMZ vs TMZ alone; key comparison pooled RT-containing arms vs TMZ alone With median follow-up 7.5 y: progression in 83.3% (10/12) on TMZ alone vs 37.5% (9/24) on RT-containing arms. PFS significantly shorter with TMZ alone: HR 3.12 (95% CI 1.26-7.69), P=0.014; adjusted HR 3.33 (95% CI 1.31-8.45), P=0.011. Median PFS 2.9 y with TMZ alone vs not reached with RT-containing arms; 3-y/5-y PFS 50%/33% vs 83%/56%. OS comparison underpowered and not significant. (jaeckle2021codelphaseiii pages 5-7, jaeckle2021codelphaseiii pages 1-2, jaeckle2021codelphaseiii pages 9-10) Neuro-Oncology (2021) Not available in retrieved context https://doi.org/10.1093/neuonc/noaa168
INDIGO (vorasidenib) Residual or recurrent grade 2 IDH1/2-mutant glioma after surgery only; included oligodendroglioma and astrocytoma; no prior RT/chemotherapy Vorasidenib 40 mg daily vs placebo 331 randomized. Median PFS 27.7 mo vs 11.1 mo; HR 0.39 (95% CI 0.27-0.56), P<0.001. Time to next intervention HR 0.26 (95% CI 0.15-0.43); median time to next intervention not reached vs 17.8 mo. Grade >=3 adverse events 16.2%-22.8% with vorasidenib vs 5.5%-13.5% with placebo across excerpts; grade >=3 ALT increase 9.6% vs 0%. About half of enrolled tumors had 1p/19q codeletion. (mellinghoff2023vorasidenibinidh1 pages 1-3, mellinghoff2023vorasidenibinidh1 pages 6-8, mellinghoff2023vorasidenibinidh1 pages 3-5, mellinghoff2023vorasidenibinidh1 pages 16-18) New England Journal of Medicine (2023) Not available in retrieved context https://doi.org/10.1056/NEJMoa2304194

Table: This table summarizes pivotal clinical trials and long-term outcome statistics relevant to IDH-mutant, 1p/19q-codeleted oligodendroglioma and related anaplastic oligodendroglial tumor populations. It is useful for comparing historical RT/PCV evidence, temozolomide-era trial data, and recent IDH-targeted therapy results.


Evidence Figure

Kaplan–Meier overall survival and progression-free survival curves for 1p/19q-codeleted subgroups comparing RT vs RT+PCV across EORTC 26951 and RTOG 9402 are shown in the joint final report figure (lassman2022jointfinalreport media 697c589d).


Notes on Evidence Gaps

Several template fields (ICD codes, MeSH/Orphanet/OMIM, population-level prevalence estimates, detailed environmental risk factors, structured HPO frequency estimates, and curated animal model resources) were not retrievable from the evidence assembled in this tool run and therefore are not asserted here.

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