ORPHA:251627
Oligodendroglioma grade 2
Also known as: Oligodendroglioma
Publications
24,026
97.8th percentile
Trials
175
Interventional, condition-specific
Researchers
1,570
Distinct authors in sample
Gene link
—
Readiness
4/6
Stages with a signal
Clinical definition (Orphanet)
A rare glial tumor characterized by a highly cellular lesion that is diffusly infiltrating at the periphery and consists of evenly-spaced monomorphic cells with the oligodendroglial . It typically occurs in the supratentorial white matter. Histologically, the cells are uniformly round to oval with round nuclei, delicate chromatin and small nucleoli. Most patients present with .
How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0016695
- MeSH:D009837
- UMLS:C0028945
- NCIT:C3288
Additional Mondo synonyms (7)
WHO grade II oligodendroglial neoplasm · WHO grade II oligodendroglial tumor · WHO grade II oligodendroglial tumour · oligodendroglioma · well differentiated oligodendroglial tumor · well differentiated oligodendroglial tumour · well differentiated oligodendroglioma
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
4/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
24,026 matched papers (14,143 in last 10 years) Source
- Phenotype characterisedPresent
1 HPO annotations (e.g. Adult onset) Source
- Animal modelPresent
5 genotype models (Mus musculus) Source
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- Interventional trialPresent
175 matched on ClinicalTrials.gov (22 recruiting in sample)
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Animal models (Monarch / Alliance)
5
Model associations linked to this Mondo ID
- Cdkn2atm1Rdp/Cdkn2a+ Tg(S100b-v-erbB)4496Waw/0 [background:] involves: 129/Sv * C57BL/6J * DBA/2J * FVB/N * SJL·MGI:3822321·Mus musculus
- Cdkn2atm1Rdp/Cdkn2atm1Rdp Tg(S100b-v-erbB)4496Waw/0 [background:] involves: 129/Sv * C57BL/6J * DBA/2J * FVB/N * SJL·MGI:3822320·Mus musculus
- Tg(S100b-v-erbB)4496Waw/0 Trp53tm1Brd/Trp53+ [background:] involves: 129S7/SvEvBrd * C57BL/6J * DBA/2J * FVB/N·MGI:3822323·Mus musculus
- Tg(S100b-v-erbB)4496Waw/0 [background:] involves: C57BL/6J * DBA/2J * FVB/N·MGI:3822319·Mus musculus
- Cdkn2atm1Rdp/Cdkn2atm1Rdp Tg(NES-TVA)J12Ech/0 [background:] involves: 129/Sv * C57BL/6 * FVB/N * SJL·MGI:3835361·Mus musculus
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
29
Drugs / clinical candidates · MONDO_0016695
- LOMUSTINE·phase 3
- PROCARBAZINE·phase 3
- SAFUSIDENIB·phase 3
- TEMOZOLOMIDE·phase 3
- VINCRISTINE·phase 3
- ABEMACICLIB·phase 2
- ALDESLEUKIN·phase 2
- BEVACIZUMAB·phase 2
- CARMUSTINE·phase 2
- CISPLATIN·phase 2
- CYCLOPHOSPHAMIDE·phase 2
- ETOPOSIDE·phase 2
- FILGRASTIM·phase 2
- MUROMONAB-CD3·phase 2
- OLAPARIB·phase 2
CTD chemicals (MyDisease.info)
9 associated chemicals · 86 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.
- Carmustine · therapeutic
- Irinotecan · therapeutic
- Lomustine · therapeutic
- PCV protocol · therapeutic
- Procarbazine · therapeutic
- Temozolomide · therapeutic
- Thiotepa · therapeutic
- Vincristine · therapeutic
- Ethylnitrosourea · marker/mechanism
Pathways: Citrate cycle (TCA cycle); Glutathione metabolism; Metabolic pathways; Carbon metabolism; 2-Oxocarboxylic acid metabolism; Biosynthesis of amino acids; Fanconi anemia pathway; Peroxisome
Literature
Is anyone studying this?
24,026
24,026 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.
24,026 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
14,143 in the last 10 years · high confidence · 97.8th percentile (publications denominator)
Phrase hits: 24,026 · MeSH hits: 519
Who's working on it?
1,570
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Brandal P4 papers · 2026
Department of Oncology, Oslo University Hospital, Oslo, Norway.
Papers in Europe PMC - 02Jakola AS4 papers · 2026
Department of Clinical Neuroscience, Institute of Neuroscience and Physiology, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Papers in Europe PMC - 03Miller JJ4 papers · 2026
Translational Neuro-Oncology Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Papers in Europe PMC - 04Abe O3 papers · 2026
Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
Papers in Europe PMC - 05Blomstrand M3 papers · 2026
Department of Oncology, Sahlgrenska University Hospital, Gothenburg, Sweden.
Papers in Europe PMC - 06Cahill DP3 papers · 2026
Translational Neuro-Oncology Laboratory, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Papers in Europe PMC - 07De Vleeschouwer S3 papers · 2026
Department of Neurosurgery, UZ Leuven, Leuven, Belgium; Department of Neurosciences and Leuven Brain Institute (LBI), KU Leuven, Leuven, Belgium.
Papers in Europe PMC - 08Duffau H3 papers · 2026
Department of Neurosurgery, Gui de Chauliac Hospital, Montpellier University Medical Center , ,
Papers in Europe PMC - 09Henssen D3 papers · 2026
Department of Medical Imaging, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands.
Papers in Europe PMC - 10Herold-Mende C3 papers · 2026
Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
175
interventional trials for this specific condition
175 interventional trials matched this specific condition name; 22 currently recruiting in our sample.
Data as of 11 September 2026
175 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 99.2th percentile).
high confidence · 99.2th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
175 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT05345002·RECRUITING·All-Trans Retinoic Acid (ATRA) Plus PD-1 Inhibition in Recurrent IDH-Mutant Glioma
Not reviewed·Conditions: Glioma · IDH Mutation · Astrocytoma · Oligodendroglioma·Matched via name + MeSH
- NCT05512351·RECRUITING·Sintilimab (One Anti-PD-1 Antibody) Plus Low-dose Bevacizumab for ctDNAlevel- Relapse and Clinical-relapse Oligodendroglioma
Not reviewed·Conditions: Oligodendroglioma·Matched via name + MeSH
- NCT05331521·RECRUITING·A Clinical Study to Improve Brain Function and Quality of Life of Patients With Newly Diagnosed Brain Tumors (Gliomas).
Not reviewed·Conditions: Oligodendroglioma·Matched via name + MeSH
- NCT06930846·RECRUITING·HOBSCOTCH-CA (HOme-Based Self-management and COgnitive Training CHanges Lives in Brain CAncer)
Not reviewed·Conditions: Brain Cancer · Brain Tumor · Brain Neoplasms · Primary Brain Tumor·Matched via name + MeSH
- NCT04623931·RECRUITING·Chemotherapy and Radiation Therapy for the Treatment of IDH Wildtype Gliomas or Non-histological (Molecular) Glioblastomas
Not reviewed·Conditions: Anaplastic Astrocytoma, IDH-Wildtype · Anaplastic Oligoastrocytoma · Anaplastic Oligodendroglioma · Diffuse Astrocytoma, IDH-Wildtype·Matched via name + MeSH
- NCT04541082·RECRUITING·Phase I Study of Oral ONC206 in Recurrent and Rare Primary Central Nervous System Neoplasms
Not reviewed·Conditions: Central Nervous System Neoplasms · Glioblastoma · Gliosarcoma, Adult · Anaplastic Oligodendroglioma·Matched via name + MeSH
- NCT01649830·RECRUITING·Efficacy of Post-radiation Adjuvant Temozolomide Chemotherapy in Residue Low-grade Glioma
Not reviewed·Conditions: Astrocytoma · Oligodendroglioma · Oligodendroastrocytoma·Matched via name + MeSH
- NCT06161974·RECRUITING·Study of Olutasidenib and Temozolomide in HGG
Not reviewed·Conditions: High Grade Glioma · Astrocytoma · Astrocytoma, Grade III · Astrocytoma, Grade IV·Matched via name + MeSH
- NCT06254326·RECRUITING·ADAGiO: Adoptive Cellular Therapy for the TreAtment of Recurrent OliGodendrogliOma (OG) Adult Patients
Not reviewed·Conditions: Recurrent Oligodendroglioma · Progressive Oligodendroglioma·Matched via name + MeSH
- NCT04702581·RECRUITING·A Randomized Trial of Delayed Radiotherapy in Patients Low-grade Oligodendrogliomas Requiring a Treatment Other Than Surgery
Not reviewed·Conditions: Oligodendroglioma · Low-grade Oligodendroglioma · 1p19q Codeletion·Matched via name + MeSH
- NCT05698524·RECRUITING·A Study of Temodar With Abexinostat (PCI-24781) for Patients With Recurrent Glioma
Not reviewed·Conditions: Recurrent High Grade Glioma · Anaplastic Astrocytoma · Anaplastic Oligodendroglioma · Glioblastoma·Matched via name + MeSH
- NCT07286292·NOT YET RECRUITING·Vorasidenib Study in Pediatric Participants With Grade 2 Astrocytoma or Oligodendroglioma With an IDH1 or IDH2 Mutation
Not reviewed·Conditions: Grade 2 Astrocytoma or Oligodendroglioma With an IDH1 or IDH2 Mutation·Matched via name + MeSH
- NCT07468136·RECRUITING·Retifanlimab With or Without Difluoromethylornithine for the Treatment of Progressive High Grade Gliomas
Not reviewed·Conditions: Anaplastic Oligodendroglioma · Astrocytoma, IDH-Mutant, Grade 3 · Astrocytoma, IDH-Mutant, Grade 4 · Diffuse Astrocytoma·Matched via name + MeSH
- NCT05190172·RECRUITING·PRO-GLIO: PROton Versus Photon Therapy in IDH-mutated Diffuse Grade II and III GLIOmas
Not reviewed·Conditions: Oligodendroglioma · Oligodendroglioma, Anaplastic · Diffuse Astrocytoma, IDH-Mutant·Matched via name + MeSH
- NCT07703436·NOT YET RECRUITING·Safety and Efficacy Study of Safusidenib in Participants With IDH1-Mutant Glioma Who Discontinued Vorasidenib Treatment Due to Progressive Disease
Not reviewed·Conditions: Grade 2 IDH1-mutant Glioma · Grade 3 IDH1-mutant Glioma · Glioma · IDH1-mutant Glioma·Matched via name + MeSH
Observational and natural-history studies
26 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
Recruiting or not-yet-recruiting
- NCT07608003·RECRUITING·Multicenter Prospective Study on MRI AI Model for Midline Glioma Subtyping and Prognosis:
Not reviewed·Conditions: Gliomas Harboring IDH1 and/or IDH2 Mutations · Glioma Glioblastoma Multiforme · Glioma of Brainstem · Glioma, Diffuse Midline, H3K27M-mutant·Matched via name + MeSH
- NCT04648462·RECRUITING·Proton Therapy Research Infrastructure- ProTRAIT- Neuro-oncology
Not reviewed·Conditions: Astrocytoma · Ependymoma · Ganglioglioma · Oligodendroglioma·Matched via name + MeSH
- NCT05536986·NOT YET RECRUITING·Correlation Between Psychological Stress and Progression of Newly Oligodendroglioma Towards Secondary Glioma
Not reviewed·Conditions: Oligodendroglioma, Adult·Matched via name + MeSH
- NCT07159607·RECRUITING·Amino Acid PET-based Response Assessment in IDH-mutant Gliomas (APPEAR)
Not reviewed·Conditions: IDH-mutant Glioma (Oligodendroglioma, Astrocytoma)·Matched via name + MeSH
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 9 · after dedupe 9 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 9 · dropped 0 · fetched 2026-07-30
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Uncertain / not reviewed (9)
- ctis·2023-509238-20-00·Cancelled·A Phase 1 Single Center Clinical Trial Evaluating Safety of 5-Aminolevulinic Acid (5-ALA) Combined With CV01 Delivery of Ultrasound for Sonodynamic Therapy (SDT) in Patients With Newly Diagnosed High-Grade Glioma (HGG) Prior to Resection and Standard Adjuvant Therapy
skipped — LLM skipped (--skip-llm)
- ctis·2024-512961-15-00·Expired·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
skipped — LLM skipped (--skip-llm)
- ctis·2023-504696-24-00·Authorised·The ALPINE trial: a phase 1-2 study of intra-arterial [177Lu]Lu-PSMA-I&T for progressive or recurrent intracranial adult type diffuse glioma
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN19852453·No longer recruiting·Radiochemotherapy trial with radiotherapy and temozolomide chemotherapy for children and young adolescents 3 years and older to 18 years of age with primary high grade glioma, pontine glioma or gliomatosis cerebri
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN45828668·No longer recruiting·A phase I - II, prospective, double blind, randomised study of the safety and efficacy of sulfasalazine for the treatment of progressing malignant gliomas
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN17931175·No longer recruiting·A joint MRC/European Organisation for Research and Treatment of Cancer (EORTC) study of radiotherapy for low grade astrocytomas and oligodendrogliomas
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN83176944·No longer recruiting·A prospective randomised trial comparing temozolomide with standard nitrosourea-based chemotherapy (PCV [procarbazine, CCNU, vincristine]/BCNU [bis-chloronitrosourea]) in the treatment of recurrent WHO astrocytic tumours grades III and IV (anaplastic astrocytoma and glioblastoma multiforme)
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN32891249·No longer recruiting·A randomised trial of adjuvant procarbazine, CCNU, vincristine chemotherapy in patients with highly anaplastic oligodendroglioma (BR11) - EORTC
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN13390479·Recruiting·The neurocognitive benefits of proton beam therapy for patients with oligodendroglioma
skipped — LLM skipped (--skip-llm)
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Oligodendroglioma grade 2 — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Oligodendroglioma grade 2" OR "Oligodendroglioma" OR "WHO grade II oligodendroglial neoplasm" OR "WHO grade II oligodendroglial tumor" OR "WHO grade II oligodendroglial tumour" OR "well differentiated oligodendroglial tumor" OR "well differentiated oligodendroglial tumour" OR "well differentiated oligodendroglioma"
MeSH descriptor terms unioned into the query: Oligodendroglioma
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Oligodendroglioma grade 2" OR "Oligodendroglioma" OR "WHO grade II oligodendroglial neoplasm" OR "WHO grade II oligodendroglial tumor" OR "WHO grade II oligodendroglial tumour" OR "well differentiated oligodendroglial tumor" OR "well differentiated oligodendroglial tumour" OR "well differentiated oligodendroglioma"
Interventional trials matched via: both (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 175 interventional · 26 observational · 1 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-27T10:50:28.169Z
