ORPHA:251663
Anaplastic oligoastrocytoma, dual genotype
Synonyms: Anaplastic oligoastrocytoma, dual genotype NEC · Anaplastic oligoastrocytoma, dual genotype, not elsewhere classified · aMOA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare and aggressive glial tumor of the central nervous system, that usually presents in adults with seizures, is most often located in the cerebral hemispheres and that is associated with a very poor prognosis.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anaplastic oligoastrocytoma, dual genotype | Anaplastic oligoastrocytoma, dual genotype NEC · Anaplastic oligoastrocytoma, dual genotype, not elsewhere classified · aMOA |
| fr | Oligoastrocytome anaplasique, génotype hybride | aMOA · Oligoastrocytome anaplasique, génotype hybride NEC · Oligoastrocytome anaplasique, génotype hybride not elsewhere classified |
| de | Anaplastisches Oligoastrozytom mit dualem Genotyp | Anaplastisches Oligoastrozytom mit doppeltem Genotyp, andernorts nicht klassifiziert · aMOA |
| es | Oligoastrocitoma anaplásico de genotipo dual | aMOA · Oligoastrocitoma anaplásico, genotipo dual, no clasificado en otra parte |
| it | Oligoastrocitoma anaplastico, genotipo ibrido | aMOA · Oligoastrocitoma anaplastico, genotipo ibrido NEC · Oligoastrocitoma anaplastico, genotipo ibrido, non classificato diversamente |
| nl | Anaplastisch oligoastrocytoom, duaal genotype | aMOA · Anaplastisch oligoastrocytoom, duaal genotype NEC · Anaplastisch oligoastrocytoom, duaal genotype, niet elders geclassificeerd |
| pt | Oligoastrocitoma anaplásico | aMOA |
| pl | Skąpodrzewiakogwiaździak anaplastyczny | aMOA |
| cs | Anaplastický oligoastrocytom | aMOA |
| uk | Анапластична олігоастроцитома | aMOA |
| zh | 间变性少突星形细胞瘤 | aMOA |
| tr | Anaplastik oligoastrositom | aMOA |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C71.9 | NTBT | index | validated |
| ICD-11 | 2A00.0Y | NTBT | index | validated |
| MONDO | 0016703 | E | validated | |
| UMLS | C0431108 | E | validated | |
| GARD | 10637 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:251651Oligoastrocytic tumor
rare neurological diseases; rare neoplastic diseases
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| IDH2 | Biomarker tested in | Assessed | HGNC:5383 | ENSG00000182054 | 147650 | 15q26.1 | isocitrate dehydrogenase (NADP(+)) 2 | 19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID] |
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Prevalence and incidence
0 recordsNo prevalence record in this release.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/251663
- This record as JSON: /data/d/251663.json