Rosette-forming glioneuronal tumor
Synonyms: RGNT
- Type
- Disease
- Group
- Disorder
- Status
- active
Rosette-forming glioneuronal tumor is a rare mixed neuronal-glial tumor characterized by the presence of uniform, rosette- (or pseudorosette-) forming neurocytes with an astrocytic component, together creating a biphasic pattern. It can present with signs of raised intracranial pressure (headache, vomiting, papilledema), hydrocephalus, seizures, ataxia and visual disturbances, or can be diagnosed incidentally in asymptomatic patients. The tumor usually arises in the midline, involving the fourth ventricle or the cerebellum.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Rosette-forming glioneuronal tumor | RGNT |
| fr | Tumeur glioneuronale formant des rosettes | = en |
| de | Tumor, glioneuronaler rosettenbildender | = en |
| es | Tumor glioneuronal formador de rosetas | = en |
| it | Tumore glioneuronale formante rosette | DNT del cervelletto · RGNT · Tumore neuroepiteliale disembrioplastico del cervelletto |
| nl | Rozetvormende glioneuronale tumor | = en |
| pt | Tumor glioneuronal formador de rosetas do quarto ventrículo | DNT do cerebelo · RGNT · Tumor neuroepitelial disembrioplásico do cerebelo |
| pl | Guz neuronalno-glejowy tworzący rozety | DNT móżdżku · Dysembrioplastyczny guz neuroepitelialny móżdżku · RGNT |
| cs | Rosetovitý glioneurální nádor čtvrté komory | RGNT · DNET mozečku · Dysembryoplastický neuroepiteliální tumor mozečku |
| uk | Розеткоподібна гліонейрональна пухлина | = en |
| zh | 玫瑰花形神经胶质瘤 | = en |
| tr | Rozet oluşturan glionöronal tümör | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.1 | NTBT | attributed | validated |
| ICD-11 | 2A00.21 | NTBT | index | validated |
| ICD-11 | XH2JU8 | E | specific | validated |
| UMLS | C4331262 | E | validated | |
| GARD | 13593 | 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/251975
- This record as JSON: /data/d/251975.json