Oligoastrocytoma

MONDO:0016702 Pathograph 5 diffuse glioma

Oligoastrocytoma is a historical, morphology-based diffuse glioma diagnosis for tumors with mixed oligodendroglial and astrocytic features. In current WHO-aligned practice, most legacy oligoastrocytomas are resolved by molecular testing into astrocytoma, IDH-mutant, or oligodendroglioma, IDH-mutant and 1p/19q-codeleted; tumors that cannot be fully classified are handled with NOS or NEC qualifiers rather than as a standalone biological entity.

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0
Mappings
0
Definitions
0
Inheritance
4
Pathophysiology
1
Histopathology
4
Phenotypes
5
Pathograph
8
Genes
4
Treatments
0
Subtypes
0
Differentials
0
Datasets
1
Trials
0
Models
1
Deep Research
🏷

Classifications

Harrison's Chapter
cancer solid tumor
⚙

Pathophysiology

4
Legacy Mixed Diffuse Glioma Morphology
The legacy diagnosis is based on diffuse glioma histology with admixed oligodendroglial-like and astrocytic-like tumor morphology. Current classification retains histology as part of integrated diagnosis but treats molecular diagnostics as central for CNS tumor classification.
oligodendrocyte link astrocyte link
cell population proliferation link ↑ INCREASED
brain link
Downstream
Molecular Reclassification Integrated molecular testing resolves most legacy mixed gliomas.
Show evidence (1 reference)
PMID:34185076 SUPPORT Human Clinical
"the 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification."
This WHO classification summary supports the entry's framing of mixed oligoastrocytoma morphology as a legacy diagnosis now interpreted through integrated molecular classification.
Molecular Reclassification
Integrated diagnosis uses IDH mutation and 1p/19q codeletion to translate most historical oligoastrocytoma diagnoses into current adult-type diffuse glioma categories. IDH-mutant tumors without whole-arm 1p/19q codeletion are generally classified as astrocytoma, IDH-mutant, while IDH-mutant tumors with whole-arm 1p/19q codeletion are classified as oligodendroglioma.
chromosome organization link ⚠ ABNORMAL
Downstream
Cortical Irritation and Mass Effect Molecularly classified diffuse gliomas remain infiltrating brain tumors.
Show evidence (1 reference)
PMID:37651614 SUPPORT Human Clinical
"The gathered data were transformed into the 2021 World Health Organization classification of CNS tumors using the IDH- and 1p/19q-mutation status."
A population registry study demonstrates that adult diffuse glioma classification in the molecular era is operationalized using IDH and 1p/19q status, directly supporting molecular reclassification of legacy diagnoses.
IDH Oncometabolic Activity
Cancer-associated IDH mutations can confer neomorphic enzymatic activity that produces the oncometabolite 2-hydroxyglutarate. This provides a mechanism-level link between IDH-mutant diffuse glioma biology and mutant IDH inhibition by vorasidenib.
metabolic process link ⚠ ABNORMAL
Downstream
Molecular Reclassification IDH-mutant oncometabolism is a defining molecular context for current classification and targeted therapy.
Show evidence (1 reference)
PMID:19935646 SUPPORT In Vitro
"cancer-associated IDH1 mutations result in a new ability of the enzyme to catalyse the NADPH-dependent reduction of alpha-ketoglutarate to R(-)-2-hydroxyglutarate (2HG)."
This biochemical and glioma study supports the oncometabolic mechanism linking IDH mutation to 2HG production.
Cortical Irritation and Mass Effect
Diffuse infiltrating glioma growth in the brain can present with headache, seizures, neurocognitive impairment, and focal neurologic deficits.
cell population proliferation link ↑ INCREASED
brain link
Show evidence (1 reference)
PMID:36809318 SUPPORT Human Clinical
"Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
This clinical review supports the neurological manifestation pattern expected from infiltrating adult brain tumors, including diffuse gliomas.
✶

Histopathology

1
Mixed Oligodendroglial and Astrocytic Morphology
Legacy oligoastrocytoma diagnosis is based on diffuse glioma histology with admixed oligodendroglial and astrocytic features, but histology alone is now insufficient for final integrated diagnosis.
Show evidence (1 reference)
PMID:34185076 SUPPORT Human Clinical
"it remains wedded to other established approaches to tumor diagnosis such as histology and immunohistochemistry."
The WHO summary supports continued use of histology within integrated CNS tumor diagnosis while emphasizing molecular classification.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Oligoastrocytoma 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

4
Nervous System 3
Seizures FREQUENT Seizure (HP:0001250)
Show evidence (1 reference)
PMID:36809318 SUPPORT Human Clinical
"Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
The cited adult brain malignancy review lists seizures among common presenting symptoms of malignant brain tumors.
Headache Headache (HP:0002315)
Show evidence (1 reference)
PMID:36809318 SUPPORT Human Clinical
"Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
The cited adult brain malignancy review lists headache among common presenting symptoms.
Neurocognitive Impairment Cognitive impairment (HP:0100543)
Show evidence (1 reference)
PMID:36809318 SUPPORT Human Clinical
"Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
The cited adult brain malignancy review lists neurocognitive impairment as a common symptom of malignant brain tumors.
Other 1
Focal Neurologic Deficits Abnormal nervous system physiology (HP:0012638)
Show evidence (1 reference)
PMID:36809318 SUPPORT Human Clinical
"Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
The cited adult brain malignancy review lists focal neurologic deficits among common symptoms. The HPO term is broader because a precise pre-coordinated term for focal neurological deficit was not available.
🧬

Genetic Associations

8
IDH1 (Somatic mutation in IDH-mutant diffuse glioma lineage)
Show evidence (2 references)
PMID:38662171 SUPPORT Human Clinical
"the presence of isocitrate dehydrogenase (IDH) mutation has been deemed the cornerstone of diagnosis."
The grade 2 glioma guideline supports IDH mutation as a diagnostic cornerstone; IDH1 is the common IDH gene in adult diffuse glioma.
PMID:19935646 SUPPORT In Vitro
"Mutations in the enzyme cytosolic isocitrate dehydrogenase 1 (IDH1) are a common feature of a major subset of primary human brain cancers."
This study supports IDH1 mutation as a recurrent feature of primary human brain cancers and supports the IDH oncometabolic mechanism.
IDH2 (Somatic mutation in IDH-mutant diffuse glioma lineage)
Show evidence (1 reference)
PMID:37272516 SUPPORT Human Clinical
"Vorasidenib, an oral brain-penetrant inhibitor of mutant IDH1 and IDH2 enzymes, showed preliminary activity in IDH-mutant gliomas."
This phase 3 clinical trial abstract directly names mutant IDH1 and IDH2 as therapeutically targetable enzymes in IDH-mutant gliomas.
1p/19q Codeletion (Chromosomal codeletion defining oligodendroglioma when paired with IDH mutation)
Show evidence (1 reference)
PMID:37651614 SUPPORT Human Clinical
"The gathered data were transformed into the 2021 World Health Organization classification of CNS tumors using the IDH- and 1p/19q-mutation status."
This registry study explicitly used IDH and 1p/19q status to map adult diffuse gliomas into 2021 WHO molecular categories.
ATRX (Somatic alteration supporting astrocytoma, IDH-mutant classification)
Show evidence (1 reference)
PMID:39315202 SUPPORT Human Clinical
"The distribution of mutations including ATRX/TP53 differed in three cohorts."
This clinical sequencing study supports ATRX and TP53 as molecular features that differ across adult-type diffuse glioma cohorts.
TP53 (Somatic alteration supporting astrocytoma, IDH-mutant classification)
Show evidence (1 reference)
PMID:39315202 SUPPORT Human Clinical
"The distribution of mutations including ATRX/TP53 differed in three cohorts."
This clinical sequencing study supports ATRX and TP53 as molecular features that differ across adult-type diffuse glioma cohorts.
TERT Promoter Mutation (Promoter mutation enriched in oligodendroglioma)
Show evidence (1 reference)
PMID:38893152 SUPPORT Human Clinical
"TERTp mutations were predominantly identified in 94.1% of oligodendrogliomas (ODG), followed by 66.3% in glioblastoma, IDH-wildtype (GBM-IDHwt), and 9.2% of astrocytomas, IDH-mutant (A-IDHm)."
This cohort study supports TERT promoter mutation as a molecular feature enriched in oligodendroglioma compared with IDH-mutant astrocytoma.
CDKN2A Homozygous Deletion (Homozygous deletion associated with adverse prognosis and molecular grade context)
Show evidence (1 reference)
PMID:38893152 SUPPORT Human Clinical
"Both uni- and multivariate analyses in GBM, age, MGMTp methylation, and CDKN2A/B homozygous deletion were statistically significant prognostic factors"
This adult-type diffuse glioma cohort supports CDKN2A/B homozygous deletion as a prognostic molecular event.
CDKN2B Homozygous Deletion (Homozygous deletion associated with adverse prognosis and molecular grade context)
Show evidence (1 reference)
PMID:38893152 SUPPORT Human Clinical
"Both uni- and multivariate analyses in GBM, age, MGMTp methylation, and CDKN2A/B homozygous deletion were statistically significant prognostic factors"
This adult-type diffuse glioma cohort supports CDKN2A/B homozygous deletion as a prognostic molecular event.
💊

Treatments

4
Maximal Safe Resection
Action: surgical procedure MAXO:0000004
Initial management typically includes maximal safe surgical resection for diagnosis, cytoreduction, symptom relief, and tissue acquisition for integrated molecular testing.
Show evidence (1 reference)
PMID:36566461 SUPPORT Human Clinical
"After histopathological diagnosis and eventually a maximal safe resection, patients with good prognostic factors may be followed by magnetic resonance imaging (MRI)."
This treatment review supports maximal safe resection as a core early management step for astrocytomas and oligodendrogliomas, the modern entities replacing most legacy oligoastrocytomas.
Radiation Therapy
Action: radiation therapy MAXO:0000014
Radiation therapy is used based on integrated molecular diagnosis, grade, residual disease, risk profile, and timing relative to chemotherapy or observation.
Show evidence (1 reference)
PMID:36566461 SUPPORT Human Clinical
"diffuse astrocytomas and oligodendrogliomas are mainly treated with combined radiochemotherapy or maximal safe resection followed by combined radiochemotherapy according to current guidelines based on randomized trials."
The treatment review supports radiotherapy as part of combined treatment for diffuse astrocytomas and oligodendrogliomas.
Alkylating Chemotherapy
Action: chemotherapy MAXO:0000647
Alkylating chemotherapy such as temozolomide or the PCV regimen is selected according to the reassigned molecular entity, tumor grade, and clinical risk profile.
Show evidence (1 reference)
PMID:36809318 SUPPORT Human Clinical
"In patients with anaplastic oligodendroglial tumors with 1p/19q codeletion, probable 20-year overall survival following radiotherapy without vs with the combination of procarbazine, lomustine, and vincristine was 13.6% vs 37.1%"
This adult brain malignancy review summarizes randomized-trial evidence for PCV chemotherapy with radiotherapy in 1p/19q-codeleted anaplastic oligodendroglial tumors, relevant to the oligodendroglioma arm of legacy oligoastrocytoma reclassification.
Vorasidenib
Action: pharmacotherapy MAXO:0000058
Agent: vorasidenib ↗
Vorasidenib is an oral brain-penetrant mutant IDH1/2 inhibitor that may delay progression and subsequent anticancer intervention in selected residual or recurrent grade 2 IDH-mutant glioma after surgery only. This is not specific to historical oligoastrocytoma but is relevant to the IDH-mutant diffuse gliomas that replaced the legacy category.
Mechanism Target:
INHIBITS IDH Oncometabolic Activity — Vorasidenib inhibits mutant IDH1/2 enzymatic activity.
Show evidence (1 reference)
PMID:37272516 SUPPORT Human Clinical
"Vorasidenib, an oral brain-penetrant inhibitor of mutant IDH1 and IDH2 enzymes, showed preliminary activity in IDH-mutant gliomas."
The INDIGO trial abstract supports vorasidenib as an inhibitor of mutant IDH1 and IDH2 enzymes.
Show evidence (1 reference)
PMID:37272516 SUPPORT Human Clinical
"In patients with grade 2 IDH-mutant glioma, vorasidenib significantly improved progression-free survival and delayed the time to the next intervention."
The phase 3 INDIGO trial directly supports vorasidenib as an IDH-directed therapy for selected grade 2 IDH-mutant gliomas.
🔬

Clinical Trials

1
NCT04164901 PHASE_III ACTIVE_NOT_RECRUITING
INDIGO/AG881-C-004 is a randomized, double-blind, placebo-controlled phase 3 trial of vorasidenib in residual or recurrent grade 2 glioma with an IDH1 or IDH2 mutation after surgery as the only prior treatment.
Show evidence (1 reference)
clinicaltrials:NCT04164901 SUPPORT Human Clinical
"Study AG881-C-004 is a phase 3, multicenter, randomized, double-blind, placebo-controlled study comparing the efficacy of vorasidenib to placebo in participants with residual or recurrent Grade 2 glioma with an IDH1 or IDH2 mutation who have undergone surgery as their only treatment."
The ClinicalTrials.gov summary directly supports the INDIGO phase 3 trial as a vorasidenib study in residual or recurrent grade 2 IDH-mutant glioma.
{ }

Source YAML

click to show 
name: Oligoastrocytoma
creation_date: "2026-05-11T12:14:16Z"
updated_date: "2026-05-11T14:24:00Z"
description: >-
  Oligoastrocytoma is a historical, morphology-based diffuse glioma diagnosis
  for tumors with mixed oligodendroglial and astrocytic features. In current
  WHO-aligned practice, most legacy oligoastrocytomas are resolved by molecular
  testing into astrocytoma, IDH-mutant, or oligodendroglioma, IDH-mutant and
  1p/19q-codeleted; tumors that cannot be fully classified are handled with NOS
  or NEC qualifiers rather than as a standalone biological entity.
categories:
- Central Nervous System Neoplasm
- Diffuse Glioma
- Adult Brain Tumor
- Molecularly Reclassified Tumor
parents:
- diffuse glioma
disease_term:
  preferred_term: oligoastrocytoma
  term:
    id: MONDO:0016702
    label: oligoastrocytoma
pathophysiology:
- name: Legacy Mixed Diffuse Glioma Morphology
  description: >-
    The legacy diagnosis is based on diffuse glioma histology with admixed
    oligodendroglial-like and astrocytic-like tumor morphology. Current
    classification retains histology as part of integrated diagnosis but treats
    molecular diagnostics as central for CNS tumor classification.
  cell_types:
  - preferred_term: oligodendrocyte
    term:
      id: CL:0000128
      label: oligodendrocyte
  - preferred_term: astrocyte
    term:
      id: CL:0000127
      label: astrocyte
  biological_processes:
  - preferred_term: cell population proliferation
    modifier: INCREASED
    term:
      id: GO:0008283
      label: cell population proliferation
  locations:
  - preferred_term: brain
    term:
      id: UBERON:0000955
      label: brain
  evidence:
  - reference: PMID:34185076
    reference_title: "The 2021 WHO Classification of Tumors of the Central Nervous System: a summary."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "the 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification."
    explanation: >-
      This WHO classification summary supports the entry's framing of mixed
      oligoastrocytoma morphology as a legacy diagnosis now interpreted through
      integrated molecular classification.
  downstream:
  - target: Molecular Reclassification
    description: Integrated molecular testing resolves most legacy mixed gliomas.
- name: Molecular Reclassification
  description: >-
    Integrated diagnosis uses IDH mutation and 1p/19q codeletion to translate
    most historical oligoastrocytoma diagnoses into current adult-type diffuse
    glioma categories. IDH-mutant tumors without whole-arm 1p/19q codeletion are
    generally classified as astrocytoma, IDH-mutant, while IDH-mutant tumors
    with whole-arm 1p/19q codeletion are classified as oligodendroglioma.
  biological_processes:
  - preferred_term: chromosome organization
    modifier: ABNORMAL
    term:
      id: GO:0051276
      label: chromosome organization
  evidence:
  - reference: PMID:37651614
    reference_title: "Epidemiology and survival of adult-type diffuse glioma in Belgium during the molecular era."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The gathered data were transformed into the 2021 World Health Organization classification of CNS tumors using the IDH- and 1p/19q-mutation status."
    explanation: >-
      A population registry study demonstrates that adult diffuse glioma
      classification in the molecular era is operationalized using IDH and
      1p/19q status, directly supporting molecular reclassification of legacy
      diagnoses.
  downstream:
  - target: Cortical Irritation and Mass Effect
    description: Molecularly classified diffuse gliomas remain infiltrating brain tumors.
- name: IDH Oncometabolic Activity
  description: >-
    Cancer-associated IDH mutations can confer neomorphic enzymatic activity
    that produces the oncometabolite 2-hydroxyglutarate. This provides a
    mechanism-level link between IDH-mutant diffuse glioma biology and mutant
    IDH inhibition by vorasidenib.
  biological_processes:
  - preferred_term: metabolic process
    modifier: ABNORMAL
    term:
      id: GO:0008152
      label: metabolic process
  evidence:
  - reference: PMID:19935646
    reference_title: "Cancer-associated IDH1 mutations produce 2-hydroxyglutarate."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "cancer-associated IDH1 mutations result in a new ability of the enzyme to catalyse the NADPH-dependent reduction of alpha-ketoglutarate to R(-)-2-hydroxyglutarate (2HG)."
    explanation: >-
      This biochemical and glioma study supports the oncometabolic mechanism
      linking IDH mutation to 2HG production.
  downstream:
  - target: Molecular Reclassification
    description: IDH-mutant oncometabolism is a defining molecular context for current classification and targeted therapy.
- name: Cortical Irritation and Mass Effect
  description: >-
    Diffuse infiltrating glioma growth in the brain can present with headache,
    seizures, neurocognitive impairment, and focal neurologic deficits.
  locations:
  - preferred_term: brain
    term:
      id: UBERON:0000955
      label: brain
  biological_processes:
  - preferred_term: cell population proliferation
    modifier: INCREASED
    term:
      id: GO:0008283
      label: cell population proliferation
  evidence:
  - reference: PMID:36809318
    reference_title: "Glioblastoma and Other Primary Brain Malignancies in Adults: A Review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
    explanation: >-
      This clinical review supports the neurological manifestation pattern
      expected from infiltrating adult brain tumors, including diffuse gliomas.
phenotypes:
- category: Neurological
  name: Seizures
  description: >-
    Seizures are a common presenting manifestation of diffuse lower-grade gliomas
    involving cerebral cortex, including tumors historically diagnosed as
    oligoastrocytoma.
  phenotype_term:
    preferred_term: Seizure
    term:
      id: HP:0001250
      label: Seizure
  frequency: FREQUENT
  evidence:
  - reference: PMID:36809318
    reference_title: "Glioblastoma and Other Primary Brain Malignancies in Adults: A Review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
    explanation: >-
      The cited adult brain malignancy review lists seizures among common
      presenting symptoms of malignant brain tumors.
- category: Neurological
  name: Headache
  description: >-
    Headache can occur from tumor mass effect, edema, or increased intracranial
    pressure in adult primary brain tumors.
  phenotype_term:
    preferred_term: Headache
    term:
      id: HP:0002315
      label: Headache
  evidence:
  - reference: PMID:36809318
    reference_title: "Glioblastoma and Other Primary Brain Malignancies in Adults: A Review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
    explanation: >-
      The cited adult brain malignancy review lists headache among common
      presenting symptoms.
- category: Neurological
  name: Neurocognitive Impairment
  description: >-
    Cognitive changes can accompany diffuse gliomas and other primary brain
    malignancies because the tumor and its treatment affect brain networks.
  phenotype_term:
    preferred_term: Neurocognitive impairment
    term:
      id: HP:0100543
      label: Cognitive impairment
  evidence:
  - reference: PMID:36809318
    reference_title: "Glioblastoma and Other Primary Brain Malignancies in Adults: A Review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
    explanation: >-
      The cited adult brain malignancy review lists neurocognitive impairment as
      a common symptom of malignant brain tumors.
- category: Neurological
  name: Focal Neurologic Deficits
  description: >-
    Focal neurological deficits may arise when infiltrating tumor affects
    eloquent cortex, white matter tracts, or other localized brain regions.
  phenotype_term:
    preferred_term: Focal neurologic deficits
    term:
      id: HP:0012638
      label: Abnormal nervous system physiology
  evidence:
  - reference: PMID:36809318
    reference_title: "Glioblastoma and Other Primary Brain Malignancies in Adults: A Review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Symptoms of malignant brain tumors include headache (50%), seizures (20%-50%), neurocognitive impairment (30%-40%), and focal neurologic deficits (10%-40%)."
    explanation: >-
      The cited adult brain malignancy review lists focal neurologic deficits
      among common symptoms. The HPO term is broader because a precise
      pre-coordinated term for focal neurological deficit was not available.
histopathology:
- name: Mixed Oligodendroglial and Astrocytic Morphology
  finding_term:
    preferred_term: Diffuse Glioma
    term:
      id: NCIT:C129325
      label: Diffuse Glioma
  description: >-
    Legacy oligoastrocytoma diagnosis is based on diffuse glioma histology with
    admixed oligodendroglial and astrocytic features, but histology alone is now
    insufficient for final integrated diagnosis.
  evidence:
  - reference: PMID:34185076
    reference_title: "The 2021 WHO Classification of Tumors of the Central Nervous System: a summary."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "it remains wedded to other established approaches to tumor diagnosis such as histology and immunohistochemistry."
    explanation: >-
      The WHO summary supports continued use of histology within integrated CNS
      tumor diagnosis while emphasizing molecular classification.
genetic:
- name: IDH1
  gene_term:
    preferred_term: IDH1
    term:
      id: hgnc:5382
      label: IDH1
  association: Somatic mutation in IDH-mutant diffuse glioma lineage
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    IDH1 mutation is a central molecular feature used to classify adult-type
    diffuse gliomas and to select IDH-directed treatment strategies.
  evidence:
  - reference: PMID:38662171
    reference_title: "SEOM-GEINO clinical guidelines for grade 2 gliomas (2023)."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "the presence of isocitrate dehydrogenase (IDH) mutation has been deemed the cornerstone of diagnosis."
    explanation: >-
      The grade 2 glioma guideline supports IDH mutation as a diagnostic
      cornerstone; IDH1 is the common IDH gene in adult diffuse glioma.
  - reference: PMID:19935646
    reference_title: "Cancer-associated IDH1 mutations produce 2-hydroxyglutarate."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "Mutations in the enzyme cytosolic isocitrate dehydrogenase 1 (IDH1) are a common feature of a major subset of primary human brain cancers."
    explanation: >-
      This study supports IDH1 mutation as a recurrent feature of primary human
      brain cancers and supports the IDH oncometabolic mechanism.
- name: IDH2
  gene_term:
    preferred_term: IDH2
    term:
      id: hgnc:5383
      label: IDH2
  association: Somatic mutation in IDH-mutant diffuse glioma lineage
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    IDH2 mutation is less common than IDH1 mutation but shares diagnostic and
    therapeutic relevance in IDH-mutant diffuse glioma.
  evidence:
  - reference: PMID:37272516
    reference_title: "Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Vorasidenib, an oral brain-penetrant inhibitor of mutant IDH1 and IDH2 enzymes, showed preliminary activity in IDH-mutant gliomas."
    explanation: >-
      This phase 3 clinical trial abstract directly names mutant IDH1 and IDH2
      as therapeutically targetable enzymes in IDH-mutant gliomas.
- name: 1p/19q Codeletion
  association: Chromosomal codeletion defining oligodendroglioma when paired with IDH mutation
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    Whole-arm 1p/19q codeletion supports oligodendroglioma classification when
    present with IDH mutation and helps distinguish oligodendroglioma from
    IDH-mutant astrocytoma in legacy mixed gliomas.
  evidence:
  - reference: PMID:37651614
    reference_title: "Epidemiology and survival of adult-type diffuse glioma in Belgium during the molecular era."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The gathered data were transformed into the 2021 World Health Organization classification of CNS tumors using the IDH- and 1p/19q-mutation status."
    explanation: >-
      This registry study explicitly used IDH and 1p/19q status to map adult
      diffuse gliomas into 2021 WHO molecular categories.
- name: ATRX
  gene_term:
    preferred_term: ATRX
    term:
      id: hgnc:886
      label: ATRX
  association: Somatic alteration supporting astrocytoma, IDH-mutant classification
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    ATRX alteration is part of the molecular context that helps distinguish
    IDH-mutant astrocytoma from oligodendroglioma in the reclassification of
    legacy mixed gliomas. The local HGNC adapter identifies ATRX as hgnc:886.
  evidence:
  - reference: PMID:39315202
    reference_title: "Clinical sequencing reveals diagnostic, therapeutic, and prognostic biomarkers for adult-type diffuse gliomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The distribution of mutations including ATRX/TP53 differed in three cohorts."
    explanation: >-
      This clinical sequencing study supports ATRX and TP53 as molecular
      features that differ across adult-type diffuse glioma cohorts.
- name: TP53
  gene_term:
    preferred_term: TP53
    term:
      id: hgnc:11998
      label: TP53
  association: Somatic alteration supporting astrocytoma, IDH-mutant classification
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    TP53 alteration is part of the astrocytoma, IDH-mutant molecular pattern
    used when resolving legacy mixed diffuse glioma diagnoses.
  evidence:
  - reference: PMID:39315202
    reference_title: "Clinical sequencing reveals diagnostic, therapeutic, and prognostic biomarkers for adult-type diffuse gliomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The distribution of mutations including ATRX/TP53 differed in three cohorts."
    explanation: >-
      This clinical sequencing study supports ATRX and TP53 as molecular
      features that differ across adult-type diffuse glioma cohorts.
- name: TERT Promoter Mutation
  gene_term:
    preferred_term: TERT
    term:
      id: hgnc:11730
      label: TERT
  association: Promoter mutation enriched in oligodendroglioma
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    TERT promoter mutation is frequent in oligodendroglioma and much less common
    in astrocytoma, IDH-mutant, adding molecular context to the 1p/19q-codeleted
    reclassification endpoint.
  evidence:
  - reference: PMID:38893152
    reference_title: "Prognostic Impact of TERT Promoter Mutations in Adult-Type Diffuse Gliomas Based on WHO2021 Criteria."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "TERTp mutations were predominantly identified in 94.1% of oligodendrogliomas (ODG), followed by 66.3% in glioblastoma, IDH-wildtype (GBM-IDHwt), and 9.2% of astrocytomas, IDH-mutant (A-IDHm)."
    explanation: >-
      This cohort study supports TERT promoter mutation as a molecular feature
      enriched in oligodendroglioma compared with IDH-mutant astrocytoma.
- name: CDKN2A Homozygous Deletion
  gene_term:
    preferred_term: CDKN2A
    term:
      id: hgnc:1787
      label: CDKN2A
  association: Homozygous deletion associated with adverse prognosis and molecular grade context
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    CDKN2A/B homozygous deletion is a grade-relevant molecular event in
    adult-type diffuse glioma classification and prognosis.
  evidence:
  - reference: PMID:38893152
    reference_title: "Prognostic Impact of TERT Promoter Mutations in Adult-Type Diffuse Gliomas Based on WHO2021 Criteria."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Both uni- and multivariate analyses in GBM, age, MGMTp methylation, and CDKN2A/B homozygous deletion were statistically significant prognostic factors"
    explanation: >-
      This adult-type diffuse glioma cohort supports CDKN2A/B homozygous
      deletion as a prognostic molecular event.
- name: CDKN2B Homozygous Deletion
  gene_term:
    preferred_term: CDKN2B
    term:
      id: hgnc:1788
      label: CDKN2B
  association: Homozygous deletion associated with adverse prognosis and molecular grade context
  relationship_type: SOMATIC_DRIVER
  variant_origin: SOMATIC
  notes: >-
    CDKN2A/B homozygous deletion is a grade-relevant molecular event in
    adult-type diffuse glioma classification and prognosis.
  evidence:
  - reference: PMID:38893152
    reference_title: "Prognostic Impact of TERT Promoter Mutations in Adult-Type Diffuse Gliomas Based on WHO2021 Criteria."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Both uni- and multivariate analyses in GBM, age, MGMTp methylation, and CDKN2A/B homozygous deletion were statistically significant prognostic factors"
    explanation: >-
      This adult-type diffuse glioma cohort supports CDKN2A/B homozygous
      deletion as a prognostic molecular event.
treatments:
- name: Maximal Safe Resection
  description: >-
    Initial management typically includes maximal safe surgical resection for
    diagnosis, cytoreduction, symptom relief, and tissue acquisition for
    integrated molecular testing.
  treatment_term:
    preferred_term: surgical procedure
    term:
      id: MAXO:0000004
      label: surgical procedure
  evidence:
  - reference: PMID:36566461
    reference_title: "Conventional and emerging treatments of astrocytomas and oligodendrogliomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "After histopathological diagnosis and eventually a maximal safe resection, patients with good prognostic factors may be followed by magnetic resonance imaging (MRI)."
    explanation: >-
      This treatment review supports maximal safe resection as a core early
      management step for astrocytomas and oligodendrogliomas, the modern
      entities replacing most legacy oligoastrocytomas.
- name: Radiation Therapy
  description: >-
    Radiation therapy is used based on integrated molecular diagnosis, grade,
    residual disease, risk profile, and timing relative to chemotherapy or
    observation.
  treatment_term:
    preferred_term: radiation therapy
    term:
      id: MAXO:0000014
      label: radiation therapy
  evidence:
  - reference: PMID:36566461
    reference_title: "Conventional and emerging treatments of astrocytomas and oligodendrogliomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "diffuse astrocytomas and oligodendrogliomas are mainly treated with combined radiochemotherapy or maximal safe resection followed by combined radiochemotherapy according to current guidelines based on randomized trials."
    explanation: >-
      The treatment review supports radiotherapy as part of combined treatment
      for diffuse astrocytomas and oligodendrogliomas.
- name: Alkylating Chemotherapy
  description: >-
    Alkylating chemotherapy such as temozolomide or the PCV regimen is selected
    according to the reassigned molecular entity, tumor grade, and clinical risk
    profile.
  treatment_term:
    preferred_term: chemotherapy
    term:
      id: MAXO:0000647
      label: chemotherapy
  evidence:
  - reference: PMID:36809318
    reference_title: "Glioblastoma and Other Primary Brain Malignancies in Adults: A Review."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "In patients with anaplastic oligodendroglial tumors with 1p/19q codeletion, probable 20-year overall survival following radiotherapy without vs with the combination of procarbazine, lomustine, and vincristine was 13.6% vs 37.1%"
    explanation: >-
      This adult brain malignancy review summarizes randomized-trial evidence
      for PCV chemotherapy with radiotherapy in 1p/19q-codeleted anaplastic
      oligodendroglial tumors, relevant to the oligodendroglioma arm of legacy
      oligoastrocytoma reclassification.
- name: Vorasidenib
  description: >-
    Vorasidenib is an oral brain-penetrant mutant IDH1/2 inhibitor that may
    delay progression and subsequent anticancer intervention in selected
    residual or recurrent grade 2 IDH-mutant glioma after surgery only. This is
    not specific to historical oligoastrocytoma but is relevant to the IDH-mutant
    diffuse gliomas that replaced the legacy category.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    therapeutic_agent:
    - preferred_term: vorasidenib
      term:
        id: NCIT:C152914
        label: Vorasidenib
  target_mechanisms:
  - target: IDH Oncometabolic Activity
    treatment_effect: INHIBITS
    description: Vorasidenib inhibits mutant IDH1/2 enzymatic activity.
    evidence:
    - reference: PMID:37272516
      reference_title: "Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma."
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: "Vorasidenib, an oral brain-penetrant inhibitor of mutant IDH1 and IDH2 enzymes, showed preliminary activity in IDH-mutant gliomas."
      explanation: >-
        The INDIGO trial abstract supports vorasidenib as an inhibitor of mutant
        IDH1 and IDH2 enzymes.
  evidence:
  - reference: PMID:37272516
    reference_title: "Vorasidenib in IDH1- or IDH2-Mutant Low-Grade Glioma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "In patients with grade 2 IDH-mutant glioma, vorasidenib significantly improved progression-free survival and delayed the time to the next intervention."
    explanation: >-
      The phase 3 INDIGO trial directly supports vorasidenib as an IDH-directed
      therapy for selected grade 2 IDH-mutant gliomas.
clinical_trials:
- name: NCT04164901
  phase: PHASE_III
  status: ACTIVE_NOT_RECRUITING
  description: >-
    INDIGO/AG881-C-004 is a randomized, double-blind, placebo-controlled phase 3
    trial of vorasidenib in residual or recurrent grade 2 glioma with an IDH1 or
    IDH2 mutation after surgery as the only prior treatment.
  evidence:
  - reference: clinicaltrials:NCT04164901
    reference_title: "A Phase 3, Multicenter, Randomized, Double-blind, Placebo-Controlled Study of AG-881 in Subjects With Residual or Recurrent Grade 2 Glioma With an IDH1 or IDH2 Mutation"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Study AG881-C-004 is a phase 3, multicenter, randomized, double-blind, placebo-controlled study comparing the efficacy of vorasidenib to placebo in participants with residual or recurrent Grade 2 glioma with an IDH1 or IDH2 mutation who have undergone surgery as their only treatment."
    explanation: >-
      The ClinicalTrials.gov summary directly supports the INDIGO phase 3 trial
      as a vorasidenib study in residual or recurrent grade 2 IDH-mutant glioma.
classifications:
  harrisons_chapter:
  - classification_value: cancer
  - classification_value: solid tumor
📚

References & Deep Research

Deep Research

1
Falcon ▸
Disease Characteristics Research Template
Edison Scientific Literature 57 citations 2026-05-11T09:28:00.999244

Question: You are an expert researcher providing comprehensive, well-cited information.

Provide detailed information focusing on: 1. Key concepts and definitions with current understanding 2. Recent developments and latest research (prioritize 2023-2024 sources) 3. Current applications and real-world implementations 4. Expert opinions and analysis from authoritative sources 5. Relevant statistics and data from recent studies

Format as a comprehensive research report with proper citations. Include URLs and publication dates where available. Always prioritize recent, authoritative sources and provide specific citations for all major claims.

Disease Characteristics Research Template

Target Disease

  • Disease Name: Oligoastrocytoma
  • MONDO ID: (if available)
  • Category:

Research Objectives

Please provide a comprehensive research report on Oligoastrocytoma covering all of the disease characteristics listed below. This report will be used to populate a disease knowledge base entry. Be thorough and cite primary literature (PMID preferred) for all claims.

For each section, suggested databases/resources are listed. These are the first places you should search for information on each topic.

1. Disease Information

Search first: OMIM, Orphanet, ICD-10/ICD-11, MeSH, PubMed

  • What is the disease? Provide a concise overview.
  • What are the key identifiers? (OMIM, Orphanet, ICD-10/ICD-11, MeSH, Mondo)
  • What are the common synonyms and alternative names?
  • Is the information derived from individual patients (e.g., EHR) or aggregated disease-level resources?

2. Etiology

  • Disease Causal Factors: What are the primary causes? (genetic, environmental, infectious, mechanistic)
  • Risk Factors:

    Search first: PubMed, Cochrane Library, UpToDate, clinical guidelines, ClinVar, ClinGen, GWAS Catalog, PheGenI, CTD, CDC, WHO, epidemiological databases

  • Genetic risk factors (causal variants, susceptibility loci, modifier genes)
  • Environmental risk factors (toxins, lifestyle, occupational exposures, age, sex, family history)
  • Protective Factors:

    Search first: PubMed, Cochrane Library, clinical trial databases, GWAS Catalog, gnomAD, WHO, CDC, nutrition databases

  • Genetic protective factors (protective variants, modifier alleles)
  • Environmental protective factors (diet, lifestyle, exposures that reduce risk)
  • Gene-Environment Interactions: How do genetic and environmental factors interact to influence disease?

    Search first: CTD, PubMed, PheGenI, GxE databases

3. Phenotypes

Search first: HPO (Human Phenotype Ontology), OMIM, Orphanet, PubMed, clinicaltrials.gov, MedDRA, SNOMED CT, DECIPHER, LOINC

For each phenotype, provide: - Phenotype type: symptoms, clinical signs, physical manifestations, behavioral changes, or laboratory abnormalities

For symptoms/signs: HPO, OMIM, Orphanet, PubMed For behavioral changes: HPO, DSM, RDoC (Research Domain Criteria), PubMed For laboratory abnormalities: LOINC, SNOMED CT, LabTests Online, PubMed - Phenotype characteristics: Search first: OMIM, Orphanet, HPO, PubMed - Age of symptom onset (neonatal, childhood, adult-onset, late-onset) - Symptom severity (mild, moderate, severe, variable) - Symptom progression (stable, progressive, episodic, fluctuating) - Frequency among affected individuals (percentage or qualitative) - Quality of life impact: Effects on daily functioning and well-being (per-phenotype when possible) Search first: EQ-5D database, SF-36, WHO QOL databases, PubMed - Suggest HPO (Human Phenotype Ontology) terms for each phenotype

4. Genetic/Molecular Information

  • Causal Genes: Gene mutations or chromosomal abnormalities responsible for disease (gene symbols, OMIM IDs)

    Search first: OMIM, ClinVar, HGMD, Ensembl, NCBI Gene

  • Pathogenic Variants:
  • Affected genes (gene symbols, HGNC IDs) > Search first: OMIM, NCBI Gene, Ensembl, HGNC, UniProt, GeneCards
  • Variant classification (pathogenic, likely pathogenic, VUS per ACMG/AMP guidelines) > Search first: ClinVar, ClinGen, ACMG/AMP guidelines, VarSome
  • Variant type/class (missense, frameshift, nonsense, splice-site, structural)
  • Allele frequency in population databases > Search first: gnomAD, 1000 Genomes, ExAC, TOPMed, dbSNP
  • Somatic vs germline origin > Search first: COSMIC (somatic), ClinVar, ICGC, TCGA
  • Functional consequences (loss of function, gain of function, dominant negative)
  • Modifier Genes: Genes that modify disease severity or expression
  • Epigenetic Information: DNA methylation, histone modifications, chromatin changes affecting disease

    Search first: ENCODE, Roadmap Epigenomics, MethBase, DiseaseMeth

  • Chromosomal Abnormalities: Large-scale genetic changes (aneuploidy, translocations, inversions)

    Search first: DECIPHER, ClinVar, ECARUCA, UCSC Genome Browser

5. Environmental Information

  • Environmental Factors: Non-genetic contributing factors (toxins, radiation, pollution, occupational exposure)

    Search first: CTD (Comparative Toxicogenomics Database), TOXNET, PubMed, EPA databases

  • Lifestyle Factors: Behavioral factors (smoking, diet, exercise, alcohol consumption)

    Search first: CDC databases, WHO, PubMed, NHANES

  • Infectious Agents: If applicable, pathogens causing or triggering disease (bacteria, viruses, fungi, parasites)

    Search first: NCBI Taxonomy, ViPR, BV-BRC, MicrobeDB, GIDEON

6. Mechanism / Pathophysiology

  • Molecular Pathways: Specific signaling cascades or biochemical pathways involved (Wnt, MAPK, mTOR, PI3K-AKT, etc.)

    Search first: KEGG, Reactome, WikiPathways, PathBank, BioCyc

  • Cellular Processes: Cell-level mechanisms (apoptosis, autophagy, cell cycle dysregulation, inflammation, etc.)

    Search first: Gene Ontology (GO), Reactome, KEGG, PubMed

  • Protein Dysfunction: How protein structure or function is altered (misfolding, aggregation, loss of function, gain of function)

    Search first: UniProt, PDB (Protein Data Bank), InterPro, Pfam, AlphaFold

  • Metabolic Changes: Alterations in metabolic processes (energy metabolism, lipid metabolism, amino acid metabolism)

    Search first: KEGG, BioCyc, HMDB (Human Metabolome Database), BRENDA

  • Immune System Involvement: Role of immune response (autoimmunity, immunodeficiency, chronic inflammation)

    Search first: ImmPort, Immunome Database, IEDB, Gene Ontology

  • Tissue Damage Mechanisms: How tissues/ are injured (oxidative stress, ischemia, fibrosis, necrosis)

    Search first: PubMed, Gene Ontology, Reactome

  • Biochemical Abnormalities: Specific molecular defects (enzyme deficiencies, receptor dysfunction, ion channel defects)

    Search first: BRENDA, UniProt, KEGG, OMIM, PubMed

  • Epigenetic Changes: DNA methylation, histone modifications affecting gene expression in disease

    Search first: ENCODE, Roadmap Epigenomics, MethBase, DiseaseMeth

  • Molecular Profiling (if available):
  • Transcriptomics/gene expression changes > Search first: GEO (Gene Expression Omnibus), ArrayExpress, GTEx, Human Cell Atlas, SRA
  • Proteomics findings > Search first: PRIDE, ProteomeXchange, Human Protein Atlas, STRING, BioGRID
  • Metabolomics signatures > Search first: MetaboLights, Metabolomics Workbench, HMDB, METLIN
  • Lipidomics alterations > Search first: LIPID MAPS, SwissLipids, LipidHome, Metabolomics Workbench
  • Genomic structural features > Search first: UCSC Genome Browser, Ensembl, NCBI, dbVar, DGV
  • Advanced Technologies (if applicable):
  • Single-cell analysis findings (cell-type specific mechanisms, cellular heterogeneity) > Search first: Human Cell Atlas, Single Cell Portal, GEO, CELLxGENE
  • Spatial transcriptomics findings > Search first: GEO, Spatial Research, Vizgen, 10x Genomics data
  • Multi-omics integration results > Search first: TCGA, ICGC, cBioPortal, LinkedOmics, PubMed
  • Functional genomics screens (CRISPR, RNAi) > Search first: DepMap, GenomeRNAi, PubMed, BioGRID ORCS

For each mechanism, describe: - The causal chain from initial trigger to clinical manifestation - Which mechanisms are upstream vs downstream - What cell types and biological processes are involved - Suggest GO terms for biological processes and CL terms for cell types

7. Anatomical Structures Affected

  • Organ Level:
  • Primary organs directly affected
  • Secondary organ involvement (complications, secondary effects)
  • Body systems involved (cardiovascular, nervous, digestive, respiratory, endocrine, etc.)

    Search first: Uberon, FMA (Foundational Model of Anatomy), OMIM, HPO, ICD-11, MeSH, SNOMED CT

  • Tissue and Cell Level:
  • Specific tissue types affected (epithelial, connective, muscle, nervous)
  • Specific cell populations targeted (with Cell Ontology terms)

    Search first: Uberon, Human Protein Atlas, Cell Ontology, Human Cell Atlas, CellMarker, PanglaoDB

  • Subcellular Level:
  • Cellular compartments involved (mitochondria, nucleus, ER, lysosomes) (with GO Cellular Component terms)

    Search first: Gene Ontology (Cellular Component), UniProt, Human Protein Atlas

  • Localization:
  • Specific anatomical sites (with UBERON terms) > Search first: FMA, Uberon, NeuroNames (for brain), SNOMED CT
  • Lateralization (unilateral, bilateral, asymmetric) > Search first: HPO, clinical literature, imaging databases

8. Temporal Development

  • Onset:
  • Typical age of onset (congenital, pediatric, adult, geriatric)
  • Onset pattern (acute, subacute, chronic, insidious)

    Search first: OMIM, Orphanet, HPO, PubMed

  • Progression:
  • Disease stages (early, intermediate, advanced, end-stage) > Search first: Cancer Staging Manual (AJCC), WHO classifications, PubMed
  • Progression rate (rapid, slow, variable)
  • Disease course pattern (episodic, relapsing-remitting, progressive, stable)
  • Disease duration (self-limited, chronic lifelong)

    Search first: Disease registries, longitudinal cohort databases, natural history studies, PubMed, Orphanet, OMIM

  • Patterns:
  • Remission patterns (spontaneous, treatment-induced) > Search first: Clinical trial databases, disease registries, PubMed
  • Critical periods (time windows of vulnerability or opportunity for intervention) > Search first: PubMed, developmental biology databases, clinical guidelines

9. Inheritance and Population

  • Epidemiology:
  • Prevalence (cases per 100,000 at given time)
  • Incidence (new cases per 100,000 per year)

    Search first: Orphanet, CDC, WHO, GBD (Global Burden of Disease), national registries, SEER, disease registries

  • For Genetic Etiology:
  • Inheritance pattern (AD, AR, X-linked, mitochondrial, multifactorial, polygenic) > Search first: OMIM, Orphanet, ClinVar, GTR (Genetic Testing Registry)
  • Penetrance (complete, incomplete, age-dependent) > Search first: ClinVar, OMIM, PubMed, ClinGen
  • Expressivity (variable, consistent) > Search first: OMIM, ClinVar, PubMed
  • Genetic anticipation (increasing severity in successive generations) > Search first: OMIM, PubMed (especially for repeat expansion disorders)
  • Germline mosaicism > Search first: ClinVar, OMIM, genetic counseling literature, PubMed
  • Founder effects (population-specific mutations) > Search first: gnomAD, population genetics databases, PubMed
  • Consanguinity role > Search first: OMIM, population studies, genetic counseling resources
  • Carrier frequency > Search first: gnomAD, carrier screening databases, GeneReviews, GTR
  • Population Demographics:
  • Affected populations (ethnic or demographic groups with higher prevalence) > Search first: gnomAD, 1000 Genomes, PAGE Study, PubMed, population registries
  • Geographic distribution (endemic areas, regional variation) > Search first: WHO, CDC, GBD, Orphanet, geographic epidemiology databases
  • Geographic distribution of specific variants
  • Sex ratio (male:female) > Search first: Disease registries, OMIM, PubMed, epidemiological databases
  • Age distribution of affected individuals > Search first: CDC, disease registries, SEER, Orphanet

10. Diagnostics

  • Clinical Tests:
  • Laboratory tests (blood, urine, tissue chemistry, specific enzyme assays) > Search first: LOINC, LabTests Online, PubMed
  • Biomarkers (proteins, metabolites, genetic markers, circulating biomarkers) > Search first: FDA Biomarker List, BEST (Biomarkers, EndpointS, and other Tools), PubMed
  • Imaging studies (X-ray, CT, MRI, PET, ultrasound) > Search first: RadLex, DICOM, Radiopaedia, imaging databases
  • Functional tests (pulmonary function, cardiac stress tests) > Search first: LOINC, clinical guidelines, PubMed
  • Electrophysiology (EEG, EMG, ECG, nerve conduction studies) > Search first: LOINC, clinical neurophysiology databases, PubMed
  • Biopsy findings (histopathology, immunohistochemistry) > Search first: SNOMED CT, College of American Pathologists resources, PubMed
  • Pathology findings (microscopic examination) > Search first: SNOMED CT, Digital Pathology databases, PubMed
  • Genetic Testing:

    Search first: GTR (Genetic Testing Registry), GeneReviews, ClinGen

  • Overview of recommended genetic testing approach
  • Whole genome sequencing (WGS) utility > Search first: GTR, ClinVar, GEL (Genomics England), gnomAD
  • Whole exome sequencing (WES) utility > Search first: GTR, ClinVar, OMIM, GeneMatcher
  • Gene panels (which panels, which genes) > Search first: GTR, ClinVar, laboratory-specific databases
  • Single gene testing > Search first: GTR, ClinVar, OMIM, GeneReviews
  • Chromosomal microarray (CMA) > Search first: DECIPHER, ClinVar, dbVar, ECARUCA
  • Karyotyping > Search first: Chromosome Abnormality Database, ClinVar, cytogenetics resources
  • FISH > Search first: ClinVar, cytogenetics databases, PubMed
  • Mitochondrial DNA testing > Search first: MITOMAP, MSeqDR, ClinVar, GTR
  • Repeat expansion testing > Search first: GTR, ClinVar, repeat expansion databases, PubMed
  • Omics-Based Diagnostics (if applicable):
  • RNA sequencing / transcriptomics > Search first: GEO, ArrayExpress, GTEx, RNA-seq databases
  • Proteomics > Search first: PRIDE, ProteomeXchange, FDA Biomarker database
  • Metabolomics > Search first: MetaboLights, Metabolomics Workbench, HMDB
  • Epigenomics > Search first: GEO, ENCODE, Roadmap Epigenomics, MethBase
  • Liquid biopsy > Search first: COSMIC, ClinVar, liquid biopsy databases, PubMed
  • Clinical Criteria:
  • Standardized diagnostic criteria (DSM, ICD, society guidelines) > Search first: DSM-5, ICD-11, clinical society guidelines, UpToDate
  • Differential diagnosis (other conditions to rule out, with distinguishing features) > Search first: DynaMed, UpToDate, clinical decision support systems
  • Screening:
  • Screening methods for asymptomatic individuals (newborn screening, carrier screening, cascade screening) > Search first: ACMG recommendations, CDC newborn screening, GTR

11. Outcome/Prognosis

  • Survival and Mortality:
  • Survival rate (5-year, 10-year, overall) > Search first: SEER, cancer registries, disease-specific registries, PubMed
  • Life expectancy (with and without treatment if applicable) > Search first: Orphanet, disease registries, actuarial databases, PubMed
  • Mortality rate > Search first: CDC, WHO, GBD, national mortality databases
  • Disease-specific mortality (deaths directly attributable to disease) > Search first: Disease registries, CDC Wonder, GBD, PubMed
  • Morbidity and Function:
  • Morbidity (disease-related disability and health impacts) > Search first: GBD, WHO, disability databases, PubMed
  • Disability outcomes (long-term functional impairments) > Search first: ICF (International Classification of Functioning), disability registries
  • Quality of life measures (EQ-5D, SF-36, PROMIS, disease-specific tools) > Search first: EQ-5D database, SF-36, PROMIS, PubMed
  • Disease Course:
  • Complications (secondary problems: infections, organ failure, etc.) > Search first: ICD codes, disease registries, clinical databases, PubMed
  • Recovery potential (likelihood and extent of recovery, with vs without treatment) > Search first: Natural history studies, rehabilitation databases, PubMed
  • Prediction:
  • Prognostic factors (age, disease severity, biomarkers, treatment response) > Search first: Prognostic models databases, clinical calculators, PubMed
  • Prognostic biomarkers (molecular markers predicting disease course) > Search first: FDA Biomarker database, PubMed, cancer prognostic databases

12. Treatment

  • Pharmacotherapy:
  • Pharmacological treatments (drug names, drug classes, mechanisms of action) > Search first: DrugBank, RxNorm, ATC classification, DailyMed, FDA databases
  • Pharmacogenomics (how genetic variants affect drug metabolism, efficacy, toxicity) > Search first: PharmGKB, CPIC (Clinical Pharmacogenetics), FDA Table of PGx Biomarkers
  • Advanced Therapeutics:
  • Gene therapy (viral vectors, CRISPR, gene replacement, gene editing) > Search first: ClinicalTrials.gov, FDA gene therapy database, ASGCT resources
  • Cell therapy (stem cell transplant, CAR-T, cellular therapeutics) > Search first: ClinicalTrials.gov, FDA cell therapy database, FACT standards
  • RNA-based therapies (ASOs, siRNA, mRNA therapies) > Search first: ClinicalTrials.gov, FDA approvals, PubMed
  • Targeted therapies (treatments directed at specific molecular targets) > Search first: My Cancer Genome, OncoKB, ClinicalTrials.gov, FDA approvals
  • Immunotherapies (checkpoint inhibitors, monoclonal antibodies) > Search first: Cancer Immunotherapy Database, FDA approvals, ClinicalTrials.gov
  • Surgical and Interventional:
  • Surgical interventions (types of surgery, timing, outcomes) > Search first: CPT codes, surgical registries, clinical guidelines, PubMed
  • Supportive and Rehabilitative:
  • Supportive care (symptom management, pain control, nutrition) > Search first: Clinical guidelines, Cochrane Library, PubMed
  • Rehabilitation (physical therapy, occupational therapy, speech therapy) > Search first: Rehabilitation medicine databases, clinical guidelines, PubMed
  • Experimental:
  • Experimental treatments in clinical trials (with NCT identifiers if available) > Search first: ClinicalTrials.gov, EU Clinical Trials Register, WHO ICTRP
  • Treatment Outcomes:
  • Treatment response rates > Search first: Clinical trial databases, FDA reviews, systematic reviews, PubMed
  • Side effects and adverse events > Search first: FDA Adverse Event Reporting System (FAERS), MedWatch, PubMed
  • Treatment Strategy:
  • Treatment algorithms (clinical pathways, decision trees) > Search first: Clinical practice guidelines, NCCN Guidelines, UpToDate
  • Combination therapies > Search first: ClinicalTrials.gov, treatment guidelines, PubMed
  • Personalized medicine approaches (genotype-guided treatment) > Search first: My Cancer Genome, CIViC, PharmGKB, precision medicine databases

For each treatment, suggest MAXO (Medical Action Ontology) terms where applicable.

13. Prevention

  • Prevention Levels:
  • Primary prevention (preventing disease occurrence: vaccination, risk factor modification) > Search first: CDC, WHO, USPSTF recommendations, Cochrane Library
  • Secondary prevention (early detection and treatment: screening programs, early intervention) > Search first: USPSTF, CDC screening guidelines, WHO
  • Tertiary prevention (preventing complications in those with disease) > Search first: Clinical guidelines, disease management protocols, PubMed
  • Immunization: Vaccine strategies (if applicable)

    Search first: CDC vaccine schedules, WHO immunization, FDA vaccine database

  • Screening and Early Detection:
  • Screening programs (population-based: newborn screening, cancer screening) > Search first: CDC screening programs, USPSTF, cancer screening databases
  • Genetic screening (carrier screening, preimplantation genetic diagnosis, prenatal testing) > Search first: ACMG recommendations, ACOG guidelines, GTR
  • Risk stratification (identifying high-risk individuals for targeted prevention) > Search first: Risk prediction models, clinical calculators, PubMed
  • Behavioral Interventions: Lifestyle modifications to reduce risk

    Search first: CDC, WHO, behavioral intervention databases, Cochrane Library

  • Counseling: Genetic counseling (risk assessment, family planning guidance)

    Search first: NSGC resources, ACMG guidelines, GeneReviews

  • Public Health:
  • Public health interventions (sanitation, vector control, health education) > Search first: CDC, WHO, public health databases, PubMed
  • Environmental interventions (reducing environmental risk factors) > Search first: EPA databases, WHO environmental health, PubMed
  • Prophylaxis: Preventive medications or procedures

    Search first: Clinical guidelines, FDA approvals, PubMed

14. Other Species / Natural Disease

  • Taxonomy: Species affected (with NCBI Taxon identifiers)

    Search first: NCBI Taxonomy

  • Breed: Specific breeds affected (with VBO identifiers if applicable)

    Search first: VBO (Vertebrate Breed Ontology)

  • Gene: Orthologous genes in other species (with NCBI Gene IDs)

    Search first: NCBI Gene

  • Natural Disease:
  • Naturally occurring disease in other species (companion animals, wildlife) > Search first: OMIA (Online Mendelian Inheritance in Animals), VetCompass, PubMed
  • Veterinary relevance and importance in animal health > Search first: OMIA, veterinary databases, PubMed
  • Comparative Biology:
  • Comparative pathology (similarities and differences across species) > Search first: OMIA, comparative pathology databases, PubMed
  • Evolutionary conservation of disease mechanisms > Search first: HomoloGene, OrthoMCL, Alliance of Genome Resources
  • Transmission (if applicable):
  • Zoonotic potential > Search first: CDC zoonotic diseases, WHO zoonoses, GIDEON
  • Cross-species susceptibility > Search first: NCBI Taxonomy, veterinary databases, PubMed

15. Model Organisms

  • Model Types:
  • Model organism type (mammalian, invertebrate, cellular, in vitro) > Search first: Alliance of Genome Resources, model organism databases
  • Specific model systems (mouse, rat, zebrafish, Drosophila, C. elegans, yeast, cell lines, organoids, iPSCs) > Search first: MGI, RGD, ZFIN, FlyBase, WormBase, SGD, ATCC, Cellosaurus
  • Induced models (drug treatment, surgical intervention, environmental manipulation) > Search first: MGI, model organism databases, PubMed
  • Genetic Models:
  • Types available (knockout, knock-in, transgenic, conditional, humanized) > Search first: MGI, IMPC, KOMP, EuMMCR, IMSR
  • Model Characteristics:
  • Phenotype recapitulation (how well model reproduces human disease features) > Search first: Model organism databases, comparative studies, PubMed
  • Model limitations (aspects of human disease not captured) > Search first: Model organism databases, PubMed, review articles
  • Applications:
  • Research applications (what aspects of disease can be studied) > Search first: Model organism databases, PubMed
  • Resources:
  • Model databases > Search first: MGI, RGD, ZFIN, FlyBase, WormBase, IMSR, EMMA, MMRRC

Citation Requirements

  • Cite primary literature (PMID preferred) for all mechanistic and clinical claims
  • Prioritize recent reviews and landmark papers
  • Include direct quotes from abstracts where possible to support key statements
  • Distinguish evidence source types: human clinical, model organism, in vitro, computational

Output Format

Structure your response as a comprehensive narrative organized by the sections above. For each section, provide: - Factual content with specific details (numbers, percentages, gene names, variant nomenclature) - Ontology term suggestions (HPO, GO, CL, UBERON, CHEBI, MAXO, MONDO) where applicable - Evidence citations with PMIDs - Direct quotes from abstracts to support key claims - Clear indication when information is not available or not applicable for this disease

This report will be used to populate a disease knowledge base entry with: - Pathophysiology descriptions with causal chains - Gene/protein annotations (HGNC, GO terms) - Phenotype associations (HP terms) with frequencies - Cell type involvement (CL terms) - Anatomical locations (UBERON terms) - Chemical entities (CHEBI terms) - Treatment annotations (MAXO terms) - Evidence items with PMIDs and exact abstract quotes - Epidemiology, prognosis, diagnostic, and prevention information - Animal model descriptions with phenotype recapitulation details

Comprehensive Disease Characteristics Report: Oligoastrocytoma

Executive summary (current understanding)

Oligoastrocytoma is a historical, morphology-based “mixed” diffuse glioma diagnosis that has been largely eliminated from routine classification because tumors with mixed oligodendroglial/astrocytic histology typically segregate into either astrocytoma, IDH-mutant or oligodendroglioma, IDH-mutant and 1p/19q-codeleted when molecular testing is performed. WHO CNS5 (2021) explicitly emphasizes molecular diagnostics to reassign “poorly defined entities (eg, oligoastrocytomas …) to more objectively defined types” and lists only three adult-type diffuse glioma types (astrocytoma IDH-mutant; oligodendroglioma IDH-mutant/1p19q-codeleted; glioblastoma IDH-wildtype). (louis2021the2021who pages 15-16, horbinski2022clinicalimplicationsof pages 2-3)

Entity / concept WHO 2016/2021 status Defining molecular features Prognosis / epidemiology highlights Standard treatment / 2023–2024 advance
Oligoastrocytoma (historical mixed glioma) Historically used for diffuse gliomas with mixed astrocytic/oligodendroglial morphology; WHO 2016 largely removed it, retaining the term only for rare unresolved cases; WHO 2021 adult diffuse gliomas are instead assigned to molecularly defined entities, with NOS/NEC labels if workup is incomplete or unclassifiable (horbinski2022clinicalimplicationsof pages 1-2, louis2021the2021who pages 15-16, horbinski2022clinicalimplicationsof pages 2-3) Mixed histology alone is no longer sufficient; molecular testing is required to reassign most tumors to astrocytoma, IDH-mutant or oligodendroglioma, IDH-mutant and 1p/19q-codeleted (reuss2023updatesonthe pages 1-2, perez2021theevolvingclassification pages 1-2, horbinski2022clinicalimplicationsof pages 2-3) Historical survival statistics are hard to interpret because many legacy “oligoastrocytomas” are now redistributed into molecular classes; contemporary population statistics should therefore be read via reclassified astrocytoma/oligodendroglioma cohorts rather than the old label (louis2021the2021who pages 15-16, pinson2024epidemiologyandsurvival pages 2-3) No modern disease-specific regimen because the entity is obsolete in routine classification; management follows the reassigned molecular entity (horbinski2022clinicalimplicationsof pages 1-2, horbinski2022clinicalimplicationsof pages 2-3)
Astrocytoma, IDH-mutant One of the 3 WHO 2021 adult-type diffuse glioma types; all IDH-mutant diffuse astrocytic tumors are grouped into a single type and graded CNS WHO 2–4 (louis2021the2021who pages 15-16, antonelli2022adulttypediffuse pages 1-2) IDH1/2 mutation with no whole-arm 1p/19q codeletion; typically ATRX loss and TP53 alteration; CDKN2A/B homozygous deletion upgrades to grade 4; TERT promoter mutation uncommon (~9.2% in one 2024 cohort) (whitfield2022classificationofadult‐type pages 4-6, martin2023fromtheoryto pages 2-4, antonelli2022adulttypediffuse pages 2-4, lee2024prognosticimpactof pages 1-2) Belgium registry 2017–2019: 3-year survival 86.0% for grade 2 and 75.7% for grade 3 IDH-mutant astrocytoma; grade 4 IDH-mutant astrocytoma median OS 25.9 months (pinson2024epidemiologyandsurvival pages 1-2). TERTp mutation was not prognostic within A-IDHm alone in a 2024 cohort (p=0.268) (lee2024prognosticimpactof pages 1-2) Maximal safe resection; observation may be reasonable for selected low-risk grade 2 cases after gross total resection; otherwise postoperative radiochemotherapy. For grade 2 disease, RT+PCV is standard based on RTOG 9802; temozolomide alone appears probably inferior to RT in IDH-mutant astrocytoma. For grade 3 disease, RT followed by 12 cycles TMZ is standard (CATNON). Progressive disease remains difficult, with limited clearly effective options beyond re-operation, re-irradiation, and chemotherapy (kessler2023conventionalandemerging pages 2-4, kessler2023conventionalandemerging pages 6-7, segura2023seomgeinoclinicalguidelines pages 4-5, vazsalgado2024seomgeinoclinicalguidelines pages 4-5)
Oligodendroglioma, IDH-mutant and 1p/19q-codeleted One of the 3 WHO 2021 adult-type diffuse glioma types; replaces most tumors historically called oligodendroglioma or mixed oligoastrocytoma when molecularly confirmed (louis2021the2021who pages 15-16, horbinski2022clinicalimplicationsof pages 2-3, antonelli2022adulttypediffuse pages 1-2) IDH1/2 mutation + whole-arm 1p/19q codeletion is defining; usually ATRX retained, TP53 wild-type pattern, frequent TERT promoter mutation (~94.1% in a 2024 cohort), recurrent CIC and FUBP1 mutations; NOTCH1 mutation may worsen OS (li2024clinicalsequencingreveals pages 1-2, whitfield2022classificationofadult‐type pages 4-6, martin2023fromtheoryto pages 4-6, lee2024prognosticimpactof pages 1-2) Belgium registry 2017–2019: oligodendroglioma incidence about 0.52/100,000 person-years; 3-year survival 93.4% for grade 2 and 64.2% for grade 3, with median survival not reached in available follow-up (pinson2024epidemiologyandsurvival pages 3-4). CBTRUS 2017–2021: oligodendroglioma 5-year relative survival 96.0% overall and 10-year relative survival 92.9% overall (price2024cbtrusstatisticalreport pages 70-72) Maximal safe resection plus risk-adapted adjuvant therapy. For grade 2/high-risk disease, RT+PCV is standard; for grade 3 disease, randomized trials (RTOG 9402, EORTC 26951) support RT+PCV, approximately doubling OS in codeleted tumors. Temozolomide is easier to administer but has not proven equivalent to PCV in this setting (kessler2023conventionalandemerging pages 2-4, kessler2023conventionalandemerging pages 6-7, segura2023seomgeinoclinicalguidelines pages 4-5, horbinski2022clinicalimplicationsof pages 4-5)
Key registry-level epidemiology context Modern epidemiology increasingly depends on molecular reclassification rather than legacy histology (pinson2024epidemiologyandsurvival pages 2-3, pinson2024epidemiologyandsurvival pages 1-2) Belgium registry reclassified 2,233 adult diffuse gliomas by WHO 2021 molecular scheme; full molecular status available for 67.1% of cases (pinson2024epidemiologyandsurvival pages 1-2) Belgium 2017–2019: overall adult diffuse glioma age-standardized incidence 8.55/100,000 person-years; grade 4 lesions 6.72/100,000 (pinson2024epidemiologyandsurvival pages 1-2). CBTRUS 2017–2021: all primary malignant + nonmalignant CNS tumors 25.34/100,000, malignant tumors 6.89/100,000; malignant brain/CNS tumor 5-year relative survival 35.7% overall (price2024cbtrusstatisticalreport pages 70-72) These data are most useful for contextualizing how rare modern oligodendroglioma/IDH-mutant astrocytoma subsets sit within the broader diffuse glioma burden (price2024cbtrusstatisticalreport pages 70-72, pinson2024epidemiologyandsurvival pages 1-2)
Notable 2023–2024 targeted advance: vorasidenib (INDIGO) Not specific to historical oligoastrocytoma, but highly relevant to the IDH-mutant diffuse gliomas that replaced it (mellinghoff2023vorasidenibinidh1 pages 1-3) Oral, brain-penetrant dual mutant IDH1/2 inhibitor; trial enrolled residual/recurrent grade 2 IDH-mutant astrocytoma or oligodendroglioma after surgery only (mellinghoff2023vorasidenibinidh1 pages 1-3, mellinghoff2023vorasidenibinidh1 pages 3-5) INDIGO phase 3 (NCT04164901): median PFS 27.7 vs 11.1 months for vorasidenib vs placebo; HR for progression/death 0.39; HR for time to next intervention 0.26. Grade ≥3 ALT elevation occurred in 9.6% vs 0% with placebo; grade ≥3 adverse events were more frequent with vorasidenib overall (mellinghoff2023vorasidenibinidh1 pages 1-3, mellinghoff2023vorasidenibinidh1 pages 6-8) Provides the first major targeted systemic option that can delay radiotherapy/chemotherapy in selected untreated grade 2 IDH-mutant glioma. Guidelines now recognize IDH inhibitors as emerging/important additions, while open questions remain for higher-grade or previously treated disease (mellinghoff2023vorasidenibinidh1 pages 1-3, vazsalgado2024seomgeinoclinicalguidelines pages 1-3)

Table: This table summarizes how the historical diagnosis oligoastrocytoma has been replaced by molecularly defined adult diffuse glioma entities, with key molecular markers, prognosis, epidemiology, and treatment implications. It is useful for translating legacy terminology into current WHO 2021 clinical and research practice.

1. Disease information

1.1 What is the disease?

  • Historical definition: “Oligoastrocytoma” referred to diffusely infiltrating gliomas with mixed oligodendroglial and astrocytic morphologic features. (horbinski2022clinicalimplicationsof pages 1-2, horbinski2022clinicalimplicationsof pages 2-3)
  • Current concept: Modern WHO-aligned practice treats “oligoastrocytoma” as largely obsolete; mixed-appearing tumors are expected to be molecularly classifiable as either IDH-mutant astrocytoma (typically IDH+TP53+ATRX constellation) or oligodendroglioma (IDH mutation + whole-arm 1p/19q codeletion, often with TERT promoter mutation). (reuss2023updatesonthe pages 1-2, horbinski2022clinicalimplicationsof pages 1-2, horbinski2022clinicalimplicationsof pages 2-3)

1.2 Key identifiers (what is available in the evidence)

Because WHO now discourages use of “oligoastrocytoma” as a distinct biological entity, contemporary resources increasingly encode the disease under molecularly defined entities rather than a standalone diagnosis. Nonetheless, registry coding and pathology datasets still contain oligoastrocytoma codes: - ICD-O morphology code (dataset table): “Oligoastrocytoma, NOS” and “Anaplastic oligoastrocytoma, NOS” listed with ICD-O 9382/3 in a CNS tumor reporting dataset. (brandner2020standardsanddatasets pages 25-27) - SEER/registry recode context: an updated SEER histology recode aligned to WHO 2016-era changes includes a “mixed glioma/oligoastrocytoma” mapping consistent with ICD-O-3 9421/3 in the excerpted table; and notes the recode reflects WHO 2016 and was effective in registry analyses beginning ~2018. (forjaz2021anupdatedhistology pages 4-4)

Not available from retrieved evidence: MONDO ID, MeSH unique identifier, ICD-10/ICD-11 code, OMIM, Orphanet identifier.

1.3 Common synonyms / alternative names

  • Mixed glioma; mixed oligodendroglial–astrocytic tumor; oligoastrocytoma NOS; anaplastic oligoastrocytoma (historical). (horbinski2022clinicalimplicationsof pages 1-2, brandner2020standardsanddatasets pages 25-27)

1.4 Evidence provenance

  • Classification statements are derived from aggregated disease-level resources (WHO CNS5 summary; Nature Reviews Neurology implications review). (louis2021the2021who pages 15-16, horbinski2022clinicalimplicationsof pages 1-2)
  • Epidemiology/survival statements include population-based registry resources (Belgian Cancer Registry; CBTRUS). (pinson2024epidemiologyandsurvival pages 1-2, price2024cbtrusstatisticalreport pages 70-72)

2. Etiology

2.1 Disease causal factors (mechanistic; molecular drivers)

In contemporary practice, tumors historically labeled oligoastrocytoma are reclassified into causal/molecular categories: - Astrocytoma, IDH-mutant: typically shows an “IDH/TP53/ATRX” molecular constellation. (reuss2023updatesonthe pages 1-2, whitfield2022classificationofadult‐type pages 4-6) - Oligodendroglioma, IDH-mutant and 1p/19q-codeleted: defined by IDH mutation plus whole-arm 1p/19q codeletion; frequently has TERT promoter mutation and recurrent CIC and FUBP1 alterations. (whitfield2022classificationofadult‐type pages 4-6, li2024clinicalsequencingreveals pages 1-2, martin2023fromtheoryto pages 4-6)

2.2 Risk factors (human epidemiology)

Evidence in the retrieved corpus is strongest at the “general glioma/malignant brain tumor” level rather than oligoastrocytoma-specific: - Ionizing radiation: A 2023 JAMA review states that “Prior exposure to ionizing radiation to the CNS… is a risk factor for brain tumors.” (schaff2023glioblastomaandother pages 3-4) - Atopy/allergies and infections (associations): The same JAMA review reports that “A history of atopic conditions… and a history of varicella-zoster virus infection are associated with lower glioma risk.” (schaff2023glioblastomaandother pages 3-4) - Lifestyle/metabolic factors (recent prospective evidence): In a large prospective Norwegian cohort (CONOR; 160,938 participants; 2,877,646 person-years; 319 incident gliomas), there were no associations between glioma risk and physical activity, alcohol, smoking, marital status, diabetes, hypertension, or metabolic syndrome; LDL showed an inverse association in men (HR per category 0.84; 95% CI 0.74–0.96). (gheorghiu2024lifestyleandmetabolic pages 1-2, gheorghiu2024lifestyleandmetabolic pages 3-5)

2.3 Protective factors

  • Evidence in this retrieval suggests atopic conditions and varicella-zoster virus infection are associated with lower glioma risk (observational association). (schaff2023glioblastomaandother pages 3-4)

2.4 Gene–environment interactions

Not specifically identified for oligoastrocytoma in the retrieved evidence. Current etiologic understanding for diffuse glioma emphasizes molecular drivers and immune-related epidemiologic associations rather than established GxE mechanisms. (schaff2023glioblastomaandother pages 3-4)

3. Phenotypes

3.1 Common clinical phenotypes (glioma-level; applicable to tumors historically labeled oligoastrocytoma)

A 2023 JAMA review provides symptom frequencies for malignant brain tumors: - “Symptoms of malignant brain tumors include headache (50%), seizures (20%–50%), neurocognitive impairment (30%–40%), and focal neurologic deficits (10%–40%).” (schaff2023glioblastomaandother pages 1-3) - It further notes: “Up to 74% of patients with lower-grade gliomas present with seizures.” (schaff2023glioblastomaandother pages 3-4)

Suggested HPO terms (examples): - Seizures: HP:0001250 - Headache: HP:0002315 - Cognitive impairment: HP:0100543 - Focal neurological deficit (broad): HP:0007325 (Focal neurological sign)

3.2 Age of onset, severity, progression, frequency

  • These diffuse gliomas are typically adult tumors; registry cohorts show diagnosis in adulthood with substantial survival differences by molecular subtype (see Outcomes/Prognosis). (pinson2024epidemiologyandsurvival pages 1-2)

3.3 Quality-of-life impact

  • Quality-of-life impact is implied by high seizure burden and neurocognitive impairment frequencies in malignant brain tumors, but QoL instrument-specific statistics (EQ-5D/SF-36/PROMIS) were not retrieved. (schaff2023glioblastomaandother pages 1-3)

4. Genetic / molecular information

4.1 Causal genes / defining alterations

  • IDH1/IDH2 mutations define IDH-mutant diffuse gliomas and are central to classification; IDH1 R132H is the most common IDH mutation and detectable by IHC; noncanonical variants also occur. (martin2023fromtheoryto pages 2-4, antonelli2022adulttypediffuse pages 2-4)
  • 1p/19q codeletion (whole-arm) defines oligodendroglioma when co-occurring with IDH mutation. (whitfield2022classificationofadult‐type pages 4-6, martin2023fromtheoryto pages 4-6)
  • ATRX loss and TP53 alterations strongly support astrocytoma IDH-mutant and can obviate 1p/19q testing in many settings. (whitfield2022classificationofadult‐type pages 4-6, martin2023fromtheoryto pages 2-4)
  • CDKN2A/B homozygous deletion is a molecular grading criterion upgrading IDH-mutant astrocytoma to grade 4 regardless of histology. (whitfield2022classificationofadult‐type pages 4-6, martin2023fromtheoryto pages 2-4)
  • TERT promoter mutation: in a 2024 cohort (n=528 adult-type diffuse gliomas) TERTp mutations were found in 94.1% of oligodendrogliomas, 66.3% of IDH-wildtype glioblastomas, and 9.2% of IDH-mutant astrocytomas. (lee2024prognosticimpactof pages 1-2)
  • CIC, FUBP1, NOTCH1: oligodendrogliomas are characterized by alterations in CIC, TERTp, FUBP1, and NOTCH1; NOTCH1 mutations were associated with worse OS in one sequencing cohort. (li2024clinicalsequencingreveals pages 1-2)

4.2 Somatic vs germline

  • The defining alterations described above are characteristic somatic tumor alterations used for integrated diagnosis in neuropathology. (martin2023fromtheoryto pages 2-4, whitfield2022classificationofadult‐type pages 4-6)

4.3 Epigenetics

  • The retrieved evidence supports epigenetic relevance indirectly through IDH mutation’s diagnostic role and use of methylation profiling in modern classification, but detailed, oligoastrocytoma-specific methylation signatures were not retrieved. (reuss2023updatesonthe pages 1-2)

5. Environmental information

  • No oligoastrocytoma-specific environmental exposures were retrieved.
  • For gliomas/malignant brain tumors broadly, the best-supported environmental risk factor in this evidence set is prior ionizing radiation exposure to the CNS. (schaff2023glioblastomaandother pages 3-4)

6. Mechanism / pathophysiology (integrated causal chain)

Because “oligoastrocytoma” is not a modern biological entity, mechanisms are best described for the two molecularly defined replacement entities.

6.1 Replacement-mechanism framework

  1. Initiating oncogenic event (upstream): IDH1/2 mutation establishes an IDH-mutant diffuse glioma lineage. (reuss2023updatesonthe pages 1-2)
  2. Lineage specification (midstream):
  3. Astrocytic lineage: IDH + TP53 + ATRX molecular constellation. (reuss2023updatesonthe pages 1-2)
  4. Oligodendroglial lineage: IDH + 1p/19q whole-arm codeletion (often + TERTp). (reuss2023updatesonthe pages 1-2)
  5. Grade progression (downstream): additional molecular events can drive more aggressive behavior; for example, CDKN2A/B homozygous deletion supports grade 4 assignment in IDH-mutant astrocytoma. (whitfield2022classificationofadult‐type pages 4-6)
  6. Clinical manifestations: seizures, headache, cognitive impairment, and focal deficits result from cortical involvement and infiltrative growth. (schaff2023glioblastomaandother pages 1-3)

Suggested GO biological process terms (examples): - Regulation of cell proliferation: GO:0042127 - DNA repair (broad): GO:0006281 - Cell differentiation: GO:0030154

Suggested Cell Ontology (CL) terms (examples; likely involved cell types): - Astrocyte: CL:0000127 - Oligodendrocyte precursor cell: CL:0002453 (often hypothesized as cells-of-origin for some gliomas; not directly evidenced here) - Microglial cell: CL:0000129 (tumor microenvironment; not directly evidenced here)

7. Anatomical structures affected

  • Primary organ/system: central nervous system, especially the brain (adult-type diffuse gliomas). (louis2021the2021who pages 15-16)

Suggested UBERON terms (examples): - Brain: UBERON:0000955 - Cerebral cortex: UBERON:0000956

8. Temporal development

  • Course is typically chronic/progressive for diffuse gliomas; grade and molecular subtype strongly influence progression and survival in real-world registries. (pinson2024epidemiologyandsurvival pages 1-2)

9. Inheritance and population

9.1 Epidemiology (recent registry statistics)

United States (CBTRUS; publication Oct 2024; diagnoses 2017–2021): - “Between 2017 and 2021, the average annual age-adjusted incidence rate (AAAIR) of all primary malignant and non-malignant brain and other CNS tumors was 25.34 per 100,000 (malignant AAAIR 6.89 and non-malignant AAAIR 18.46).” (price2024cbtrusstatisticalreport pages 70-72) - Gliomas accounted for 22.9% of all tumors in CBTRUS (histology-based grouping). (price2024cbtrusstatisticalreport pages 70-72) - For oligodendroglioma (histology grouping), 5-year relative survival 96.0% overall; 10-year relative survival 92.9% overall. (price2024cbtrusstatisticalreport pages 70-72)

Belgium (Belgian Cancer Registry; publication Aug 2024; incidence window 2017–2019; reclassified to WHO 2021 molecular scheme): - Age-standardized incidence rate: 8.55 per 100,000 person-years for adult-type diffuse glioma; 6.72 per 100,000 person-years for grade 4 lesions. (pinson2024epidemiologyandsurvival pages 1-2) - Estimated incidence (ESR): grade 2 astrocytoma 0.60/100,000; grade 3 astrocytoma 0.48/100,000; oligodendroglioma ~0.52/100,000. (pinson2024epidemiologyandsurvival pages 3-4)

9.2 Demographics

  • In Belgium registry diffuse gliomas (2017–2019), median age ~64 and ~40% female (contextual registry description). (pinson2024epidemiologyandsurvival pages 4-6)

9.3 Genetic inheritance

  • For malignant brain tumors broadly, fewer than 5% report family history/predisposition syndromes in the JAMA review. (schaff2023glioblastomaandother pages 3-4)

10. Diagnostics

10.1 Diagnostic approach (WHO CNS5 integrated diagnosis)

Core tests for reclassifying “mixed” diffuse gliomas include: - IDH mutation testing (IHC for IDH1 R132H plus sequencing when needed). (martin2023fromtheoryto pages 2-4) - ATRX IHC (loss supports astrocytoma, IDH-mutant). (whitfield2022classificationofadult‐type pages 4-6) - 1p/19q codeletion testing (required to define oligodendroglioma with IDH mutation; performed if ATRX is retained). (martin2023fromtheoryto pages 2-4) - Additional molecular grading markers such as CDKN2A/B homozygous deletion. (whitfield2022classificationofadult‐type pages 4-6)

WHO CNS5 adult diffuse glioma diagnostic algorithm (visual): see the retrieved figure integrating morphology with IDH/ATRX/1p19q and grade-defining features. (martin2023fromtheoryto media fe0dadc9)

10.2 NOS/NEC usage

Where molecular workup is incomplete/unavailable, WHO encourages NOS/NEC qualifiers rather than defaulting to obsolete mixed categories. (horbinski2022clinicalimplicationsof pages 2-3)

11. Outcome / prognosis

11.1 Real-world survival (Belgium registry; 2017–2019; publication 2024)

  • IDH-wildtype glioblastoma median OS 9.3 months vs grade 4 IDH-mutant astrocytoma median OS 25.9 months. (pinson2024epidemiologyandsurvival pages 1-2)
  • 3-year survival probabilities:
  • IDH-mutant astrocytoma grade 2: 86.0%; grade 3: 75.7%. (pinson2024epidemiologyandsurvival pages 1-2)
  • Oligodendroglioma (IDH-mutant/1p19q-codeleted): grade 2 93.4%; grade 3 64.2%. (pinson2024epidemiologyandsurvival pages 3-4)

11.2 Prognostic molecular markers (recent cohort data)

  • TERT promoter mutations are frequent in oligodendroglioma but were not prognostic within oligodendroglioma in one study due to near ubiquity; in a combined astrocytic-tumor grouping, TERTp was an adverse prognostic factor, but not within IDH-mutant astrocytoma alone. (lee2024prognosticimpactof pages 1-2)
  • CDKN2A/B homozygous deletion is associated with worse outcome and is a WHO CNS5 grade-defining event in IDH-mutant astrocytoma. (whitfield2022classificationofadult‐type pages 4-6)

12. Treatment

12.1 Standard-of-care (current real-world implementations)

Across modern guidelines and reviews, treatment is determined by the reassigned molecular entity (astrocytoma IDH-mutant vs oligodendroglioma IDH-mutant/1p19q-codeleted) and grade: - Maximal safe resection is a foundational step but is not curative in grade 2 diffuse gliomas; “grade 2 gliomas are incurable by surgery and complementary treatments are vital to improving prognosis.” (SEOM-GEINO; publication Apr 2024). (vazsalgado2024seomgeinoclinicalguidelines pages 1-3) - Radiotherapy + chemotherapy are guideline-based standards: - RT + PCV has become a standard for appropriate patients (e.g., RTOG 9802 evidence base) and is considered standard-of-care in modern reviews. (kessler2023conventionalandemerging pages 2-4, vazsalgado2024seomgeinoclinicalguidelines pages 4-5) - For grade 3 IDH-mutant astrocytoma: RT followed by maintenance temozolomide is guideline-supported standard-of-care; for grade 3 oligodendroglioma: RT + PCV is standard based on randomized trials. (segura2023seomgeinoclinicalguidelines pages 4-5) - Limited options at progression: despite standard multimodality therapy, progressive disease remains difficult to treat with limited clearly effective strategies beyond re-operation, re-irradiation, and additional chemotherapy. (kessler2023conventionalandemerging pages 1-2, kessler2023conventionalandemerging pages 6-7)

Suggested MAXO terms (examples): - Surgical resection: MAXO:0000010 - Radiation therapy: MAXO:0000127 - Chemotherapy: MAXO:0000647

12.2 Targeted therapy advance (prioritize 2023–2024): Vorasidenib (INDIGO)

A major late-2023 development was the phase 3 INDIGO trial of the oral brain-penetrant mutant IDH1/2 inhibitor vorasidenib for grade 2 IDH-mutant glioma after surgery only.

Direct abstract quote (NEJM, publication Aug 2023): - “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)

Key results: - Population: residual/recurrent grade 2 IDH-mutant glioma; no prior RT/chemotherapy; n=331. (mellinghoff2023vorasidenibinidh1 pages 1-3) - PFS: 27.7 vs 11.1 months; HR 0.39 (95% CI 0.27–0.56). (mellinghoff2023vorasidenibinidh1 pages 1-3, mellinghoff2023vorasidenibinidh1 pages 6-8) - Time to next intervention: HR 0.26 (95% CI 0.15–0.43). (mellinghoff2023vorasidenibinidh1 pages 1-3, mellinghoff2023vorasidenibinidh1 pages 6-8) - Safety: grade ≥3 ALT increase ~9.6% (vorasidenib) vs 0% (placebo). (mellinghoff2023vorasidenibinidh1 pages 1-3) - Trial registry: NCT04164901. (mellinghoff2023vorasidenibinidh1 pages 1-3)

Clinical implementation (2024 guideline context): SEOM-GEINO grade 2 glioma guidelines describe IDH inhibitors as new treatments showing significant efficacy in grade 2 gliomas without prior RT/chemotherapy and reference vorasidenib in this setting. (vazsalgado2024seomgeinoclinicalguidelines pages 1-3, vazsalgado2024seomgeinoclinicalguidelines pages 4-5)

13. Prevention

There are no established primary-prevention interventions specific to oligoastrocytoma, and “modifiable” risk factor evidence for glioma is generally weak. - Recent prospective evidence supports that common lifestyle/metabolic factors largely do not materially alter glioma risk (CONOR cohort). (gheorghiu2024lifestyleandmetabolic pages 1-2, gheorghiu2024lifestyleandmetabolic pages 3-5) - The most established preventable exposure in this evidence is therapeutic/medical ionizing radiation to the CNS; prevention therefore focuses on minimizing unnecessary radiation exposure consistent with general radiological safety principles. (schaff2023glioblastomaandother pages 3-4)

14. Other species / natural disease

Not retrieved in the current evidence corpus for oligoastrocytoma specifically.

15. Model organisms / model systems

Because oligoastrocytoma is obsolete as a molecular category, model systems are typically aligned to glioblastoma or to IDH-mutant astrocytoma/oligodendroglioma biology.

A 2024 systematic review of glioma stem cell (GSC) models provides quantitative data on commonly used in vitro systems: - Included 65 studies; most common model cell lines: U87 (20 studies; 32.0%), U251 (13; 20.0%), A172 (4; 6.2%), T98G (2; 3.17%). (agosti2024gliomastemcells pages 1-2) - The review notes GSCs can “recapitulat[e] the heterogeneity of the original tumor when transplanted into immunocompromised mice,” indicating frequent xenograft use. (agosti2024gliomastemcells pages 1-2) - It also emphasizes a field shift toward more representative patient-derived xenografts (PDXs) and primary glioma cell lines because highly passaged lines like U87 do not capture tumor heterogeneity. (agosti2024gliomastemcells pages 15-17)

URLs and publication dates (selected high-authority sources used)

  • WHO CNS5 summary (Neuro-Oncology): https://doi.org/10.1093/neuonc/noab106 (published Jun 2021). (louis2021the2021who pages 15-16)
  • WHO CNS5 clinical implications review (Nat Rev Neurol): https://doi.org/10.1038/s41582-022-00679-w (published Jun 2022). (horbinski2022clinicalimplicationsof pages 1-2)
  • Belgian Cancer Registry molecular-era epidemiology (Neuro-Oncology): https://doi.org/10.1093/neuonc/noad158 (published Aug 2024). (pinson2024epidemiologyandsurvival pages 1-2)
  • CBTRUS 2017–2021 statistical report (Neuro-Oncology): https://doi.org/10.1093/neuonc/noae145 (published Oct 2024). (price2024cbtrusstatisticalreport pages 70-72)
  • Vorasidenib INDIGO phase 3 (NEJM): https://doi.org/10.1056/NEJMoa2304194 (published Aug 2023). (mellinghoff2023vorasidenibinidh1 pages 1-3)
  • SEOM-GEINO grade 2 glioma guideline (Clin Transl Oncol): https://doi.org/10.1007/s12094-024-03456-x (published Apr 2024). (vazsalgado2024seomgeinoclinicalguidelines pages 1-3)
  • Adult malignant brain tumor clinical review (JAMA): https://doi.org/10.1001/jama.2023.0023 (published Feb 2023). (schaff2023glioblastomaandother pages 1-3)

Key limitations of this report (evidence gaps)

  • A MONDO ID, MeSH identifier, ICD-10/ICD-11 codes, OMIM, and Orphanet identifiers for oligoastrocytoma were not found in the retrieved documents.
  • PMIDs were not available in the retrieved text chunks (DOIs/URLs provided instead).
  • Several requested detailed subsections (e.g., comprehensive differential diagnosis lists; detailed methylome classes; multi-omics signatures; validated QoL instrument statistics; natural disease in other species) were not available in the retrieved evidence and would require additional targeted retrieval from specialized databases and primary cohort publications.

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