RARE DISEASERESEARCH ATLAS

ORPHA:251674

Chordoid glioma

low confidenceDisorder

Publications

736

Trials

2

Interventional, condition-specific

Researchers

1,292

Distinct authors in sample

Gene link

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare central nervous system tumor characterized by a non-infiltrative and well-circumscribed WHO grade 2 glial neoplasm occurring in the third ventricle, often extending to hypothalamus. Chordoid glioma presents most frequently in middle-aged women with symptoms of memory deficits, headaches and visual loss, related to its location. Virtually all chordoid gliomas exhibit positivity for GFAP and TTF1 immunostaining, PRKCAp.D463H mutation, and a specific chordoid glioma DNA methylation class.

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

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

    736 matched papers (391 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?

736

736 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.

736 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).

391 in the last 10 years · low confidence

Phrase hits: 736 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,292

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
    Perry A6 papers · 2025

    Department of Pathology, University of California, San Francisco, CA, USA.

    Papers in Europe PMC
  2. 02
    Solomon DA6 papers · 2025

    Department of Pathology, University of California, San Francisco, CA, USA.

    Papers in Europe PMC
  3. 03
    Bielle F5 papers · 2026

    *Neuropathology Department Raymond-Escourolle ***AP-HP, Neurology 2 Mazarin Department, Pitié-Salpêtrière Hospital §§§Onconeurotek, Pitié-Salpêtrière Hospital ††Pathology Department, Hôpital Lariboisière, Assistance publique - Hôpitaux de Paris †Sorbonne Universités, UPMC Univ Paris 06, UM 75 ‡Institut National de la Santé et de la Recherche Médicale, U 1127 §Centre National de la Recherche Scientifique, UMR 7225, ICM ∥Brain and Spine Institute #Institut National de la Santé et de la Recherche Médicale, U1016, Institut Cochin, Université Paris Descartes, Paris ¶Department of Anatomical and Cytological Pathology, Hôpital Foch, Suresnes **Pathology Department, Hôpital Bretonneau, CHRU Tours, Tours ‡‡Pathology and Neuropathology Department, Centre de Biologie et Pathologie Est, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron §§Pathology Department, Plateau technique de Biologie, CHU, Dijon ∥∥Department of Pathology, Caen University Hospital ¶¶Centre National de la Recherche Scientifique, UMR 6301 ISTCT CERVOxy Group, Caen ##Pathology Department, CHU Besançon, Besançon †††Pathology and Neuropathology Department, Assistance Publique-Hôpitaux de Marseille, AP-HM CHU, Timone ‡‡‡Aix-Marseille university, INSERM U911, Marseille, France.

    Papers in Europe PMC
  4. 04
    Capper D5 papers · 2025

    Department of Neuropathology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.

    Papers in Europe PMC
  5. 05
    Sanson M5 papers · 2026

    Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Univ Paris 06 UMR S 1127, ICM, F-75013, Paris, France. marc.sanson@aphp.fr.

    Papers in Europe PMC
  6. 06
    Aldape K4 papers · 2025

    Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, MD, 20814, USA. kenneth.aldape@nih.gov.

    Papers in Europe PMC
  7. 07
    Brat DJ4 papers · 2025

    Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

    Papers in Europe PMC
  8. 08
    Kim K4 papers · 2024

    Veterinary Health Center, University of Missouri, Colombia , MO ,

    Papers in Europe PMC
  9. 09
    Pfister SM4 papers · 2025

    Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.

    Papers in Europe PMC
  10. 10
    Polivka M4 papers · 2018

    Department of Pathology, AP-HP, Hôpital Lariboisière, F-75010, 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.

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.

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 Chordoid 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.

"Chordoid glioma"

Run this search on Europe PMC

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

"Chordoid glioma"

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.

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

Ingested 2026-07-27T10:52:16.263Z