Dysembryoplastic neuroepithelial tumor
Synonyms: DNET
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
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 seizures. 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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Dysembryoplastic neuroepithelial tumor | DNET |
| fr | Tumeur neuroépithéliale dysembryoplasique | = en |
| de | Tumor, dysembryoplastischer neuroepithelialer | = en |
| es | Tumor neuroepitelial disembrioplásico | = en |
| it | Tumore neuroepiteliale disembrioplastico | = en |
| nl | Dysembryoplastische neuro-epitheliale tumor | = en |
| pt | Tumor neuroepitelial disembrioplásico | = en |
| pl | Dysembrioplastyczny nowotwór neuroepitelialny | = en |
| cs | Dysembryoplastický neuroepiteliální nádor | = en |
| uk | Дизембріопластична нейроепітеліальна пухлина | = en |
| zh | 胚胎发育不良性神经上皮瘤 | = en |
| tr | Disembriyoplastik nöroepitelyal tümör | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.0 | NTBT | attributed | not yet validated |
| ICD-11 | 2A00.21 | NTBT | index | validated |
| ICD-11 | XH0H76 | E | specific | validated |
| MONDO | 0005505 | E | validated | |
| UMLS | C1266177 | E | validated | |
| GARD | 10640 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:251934Mixed neuronal-glial tumor
rare neurological diseases; rare neoplastic diseases - ORPHA:101998Rare epilepsy
rare neurological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/251946
- This record as JSON: /data/d/251946.json