ORPHA:251946
Dysembryoplastic neuroepithelial tumor
Also known as: DNET
Publications
2,007
Trials
1
Interventional, condition-specific
Researchers
1,407
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare mixed neuronal-glial tumor characterized by a benign, usually supratentorial lesion with predominantly cortical location and multinodular architecture. The tumor typically becomes symptomatic in the second or third decade of life with drug-resistant partial . Histological hallmark is the specific glioneuronal element, columns oriented perpendicularly to the cortical surface, formed by bundles of axons attached to oligodendroglia-like cells, while neurons appear to float in an abundant eosinophilic matrix.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0005505
- UMLS:C1266177
- NCIT:C9505
Additional Mondo synonyms (4)
DNT · dysembryoplastic neuroepithelial neoplasm · dysembryoplastic neuroepithelial tumor (morphologic abnormality) · dysembryoplastic neuroepithelial tumour (morphologic abnormality)
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.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
2,007 matched papers (1,257 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPresent
1 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)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
2,007
2,007 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=5449) is 41.
2,007 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
1,257 in the last 10 years · low confidence
Phrase hits: 2,007 · MeSH hits: 0
Who's working on it?
1,407
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.
- 01
- 02Acosta AM4 papers · 2026
Department of Pathology, Indiana University School of Medicine, Indianapolis, USA.
Papers in Europe PMC - 03
- 04Coras R3 papers · 2025
Institute of Neuropathology, University of Erlangen, Erlangen, Germany.
Papers in Europe PMC - 05Feucht M3 papers · 2025
Center for Rare and Complex Childhood Onset Epilepsies, Member of ERN EpiCARE, Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.
Papers in Europe PMC - 06Kim SH3 papers · 2026
Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. paxco@yuhs.ac.
Papers in Europe PMC - 07Kim SK3 papers · 2026
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul, Republic of Korea.
Papers in Europe PMC - 08Nobusawa S3 papers · 2021
Department of Human Pathology, Gunma University Graduate School of Medicine, Gunma, Japan.
Papers in Europe PMC - 09Phi JH3 papers · 2026
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. phijh@snu.ac.kr.
Papers in Europe PMC - 10Sato K3 papers · 2026
Department of Basic Pathology, National Defense Medical College, Tokorozawa, Saitama, Japan.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
1
interventional trials for this specific condition
1 interventional trial matched this specific condition name; none in our sample are currently recruiting.
Data as of 27 July 2026
1 interventional trial — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 76.8th percentile).
low confidence · 76.8th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
1 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.
Observational and natural-history studies
1 observational study matches 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
- NCT06915649·RECRUITING·Exploration and Evaluation of Amygdalo-Hippocampectomy According to Prof. Coubes' Technique: An Anatomical, Clinical, and Educational Approach
Conditions: Hippocampal Sclerosis · Dysembryoplastic Neuroepithelial Tumor · Cortical Dysplasia · Ganglioglioma·Matched via name phrase
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
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.
"Dysembryoplastic neuroepithelial tumor" OR "dysembryoplastic neuroepithelial neoplasm" OR "dysembryoplastic neuroepithelial tumor (morphologic abnormality)" OR "dysembryoplastic neuroepithelial tumour (morphologic abnormality)"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Dysembryoplastic neuroepithelial tumor" OR "dysembryoplastic neuroepithelial neoplasm" OR "dysembryoplastic neuroepithelial tumor (morphologic abnormality)" OR "dysembryoplastic neuroepithelial tumour (morphologic abnormality)"
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: 1 interventional · 1 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
Synonyms dropped by stoplist: DNET; DNT
Confidence reasoning
- Preferred label is multi-word and distinctive
- 2 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
- Publication count (2007) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T10:56:07.592Z