Papillary glioneuronal tumor
Synonyms: PGNT · Pseudopapillary ganglioglioneurocytoma · Pseudopapillary neurocytoma with glial differentiation
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare mixed neuronal-glial tumor characterized by a supratentorial space-occupying lesion in periventricular location, often with prominent cystic change. The histological hallmark of this low-grade neoplasm is its pseudopapillary appearance with a single layer of cuboidal cells around hyalinized blood vessels, associated with sheets or focal collections of neuronal cells. Clinical presentation is variable and non-specific, most frequently with headache and seizures. Prognosis is favorable after complete resection.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Papillary glioneuronal tumor | PGNT · Pseudopapillary ganglioglioneurocytoma · Pseudopapillary neurocytoma with glial differentiation |
| fr | Tumeur glioneuronale papillaire | Ganglioglioneurocytoma pseudopapillaire · Neurocytome pseudopapillaire à différenciation gliale · PGNT |
| de | Tumor, glioneuraler papillärer | Ganglioglioneurozytom, pseudopapilläres · Neurozytom, pseudopapilläres, mit glialer Differenzierung · PGNT |
| es | Tumor glioneural papilar | Ganglioglioneurocitoma pseudopapilar · Neurocitoma pseudopapilar con diferenciación glial · PGNT |
| it | Tumore glioneurale papillare | Ganglioglioneurocitoma pseudopapillare · Neurocitoma pseudopapillare con differenziazione gliale · PGNT |
| nl | Papillaire glioneuronale tumor | PGNT · Pseudopapillair ganglioglioneurocytoom · Pseudopapillair neurocytoom met gliale differentiatie |
| pt | Tumor glioneuronal papilar | Ganglioglioneurocitoma pseudopapilar · Neurocitoma pseudopapilar com diferenciação glial · PGNT |
| pl | Guz brodawkowaty glioneuronalny | = en |
| cs | Papilární glioneurální tumor | PGNT · Pseudopapilární ganglioglioneurocytom · Pseudopapilární neurocytom s gliální diferenciací |
| uk | Папілярна гліоневрональна пухлина | PGNT · Псевдопапілярна гангліогліоневроцитома · Псевдопапілярна нейроцитома з гліальним диференціюванням |
| zh | 乳头状神经胶质瘤 | 假乳头神经细胞瘤伴胶质分化 |
| tr | Papiller glionöronal tümör | PGNT · Psödopapiller ganglioglionörositoma · Glial farklılaşma ile seyreden psödopapiller nörositoma |
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 | XH3XU4 | E | specific | validated |
| MONDO | 0016735 | E | validated | |
| UMLS | C2985174 | 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 (1):
- ORPHA:251934Mixed neuronal-glial tumor
rare neurological diseases; rare neoplastic 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/251962
- This record as JSON: /data/d/251962.json