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ORPHA:251671

Angiocentric glioma

low confidenceDisorder

Publications

776

Trials

2

Interventional, condition-specific

Researchers

1,382

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare diffuse glial neoplasm of the central nervous system characterized by a slowly progressing, grade 1 tumor usually arising in the cerebral cortex and/or brainstem, with tumor cells focally organized around the blood vessels in a distinctive pattern. Angiocentric glioma typically affects young patients and is clinically characterized by refractory and headaches. Nearly all angiocentric gliomas exhibit MYB alterations (most commonly MYB::QKI fusion). Most cases are cured by surgical resection alone and the prognosis is usually favorable.

How rare: <1 / 1 000 000 — fewer than one in a million. In a city the size of Kolkata, that might mean on the order of fifteen people.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (4)

Monomorphus angiocentric glioma · angiocentric glioma (WHO grade I) · angiocentric neuroepithelial tumor · angiocentric neuroepithelial tumour

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.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    776 matched papers (586 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    2 matched on ClinicalTrials.gov

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.

Phenotypes (Monarch / HPO)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

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

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

776

776 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

776 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).

586 in the last 10 years · low confidence

Phrase hits: 776 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,382

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.

  1. 01
    Zhang J7 papers · 2025

    Innovation Center for Cancer Research, Clinical Oncology School of Fujian Medical University, Fujian Cancer Hospital, Fuzhou 350014, PR China.

    Papers in Europe PMC
  2. 02
    Zhang Y6 papers · 2026

    Department of Radiation Oncology, Anhui No. 2 Provincial People's Hospital, Hefei, 230031, China.

    Papers in Europe PMC
  3. 03
    Dangouloff-Ros V5 papers · 2026

    Pediatric Radiology Department, Assistance Publique Hôpitaux de Paris, Hôpital Necker Enfants Malades and INSERM ERL UA10, INSERM U1163, Institut Imagine, Paris, France.

    Papers in Europe PMC
  4. 04
    Grill J5 papers · 2026

    Department of Pediatric and Adolescent Oncology, Gustave Roussy,, Villejuif, France

    Papers in Europe PMC
  5. 05
    Tauziède-Espariat A5 papers · 2025

    Service d'anatomie et cytologie pathologiques, hôpital Lariboisière, 2, rue Ambroise-Paré, 75475 Paris, France. Electronic address: arnault.tauziedeespariat@gmail.com.

    Papers in Europe PMC
  6. 06
    Varlet P5 papers · 2026

    Department of Neuropathology, Paris, France

    Papers in Europe PMC
  7. 07
    Boddaert N4 papers · 2026

    Pediatric Radiology Department, Assistance Publique Hôpitaux de Paris, Hôpital Necker Enfants Malades and INSERM ERL UA10, INSERM U1163, Institut Imagine, Paris, France.

    Papers in Europe PMC
  8. 08
    Li X4 papers · 2026

    Department of Radiation Oncology, Anhui No. 2 Provincial People's Hospital, Hefei, 230031, China.

    Papers in Europe PMC
  9. 09
    Wang Y4 papers · 2026

    Translational Medical Center, Weifang No. 2 People's Hospital, Weifang Respiratory Disease Hospital, Weifang, Shandong 261041, P.R. China.

    Papers in Europe PMC
  10. 10
    Beccaria K3 papers · 2026

    Pediatric Neurosurgery, Assistance Publique Hôpitaux de Paris, Hôpital Necker Enfants Malades, Université Paris Cité, Paris, France.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

2

interventional trials for this specific condition

2 interventional trials matched this specific condition name; none in our sample are currently recruiting. 1,055 trials are registered for glioma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

2 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 84.5th percentile).

low confidence · 84.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

2 interventional trials matched after quoted-phrase search and title/condition post-filter.

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Broader category: glioma

1,055

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

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 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · 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).

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 Angiocentric glioma — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This 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.

"Angiocentric glioma" OR "Monomorphus angiocentric glioma" OR "angiocentric glioma (WHO grade I)" OR "angiocentric neuroepithelial tumor" OR "angiocentric neuroepithelial tumour"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Angiocentric glioma" OR "Monomorphus angiocentric glioma" OR "angiocentric glioma (WHO grade I)" OR "angiocentric neuroepithelial tumor" OR "angiocentric neuroepithelial tumour"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 2 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"glioma"

Query health: ok — strategies attempted: phrase; with hits: phrase

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
  • Publication count (776) is implausibly high for prevalence class "<1 / 1 000 000" — treat as possible over-matching, not a measure of research intensity

Ingested 2026-07-27T10:52:07.507Z