Oligodendroglioma grade 3
Synonyms: Anaplastic oligodendroglioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare glial tumor characterized by a grade III oligodendroglial tumour with focal or diffuse anaplastic features. It typically occurs in the supratentorial white matter. Histologically, the cells are enlarged and epithelioid with pleomorphic and increased size nuclei, a vesicular chromatin pattern and prominent nucleoli. Most patients present with seizures.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Oligodendroglioma grade 3 | Anaplastic oligodendroglioma |
| fr | Oligodendrogliome grade 3 | Oligodendrogliome anaplasique |
| de | Oligodendrogliom Grad 3 | Oligodendrogliom, anaplastisches |
| es | Oligodendroglioma de grado 3 | Oligodendroglioma anaplásico |
| it | Oligodendroglioma anaplastico | |
| nl | Oligodendroglioom graad 3 | Anaplastisch oligodendroglioom |
| pt | Oligodendroglioma anaplásico | |
| pl | Skapodrzewiak anaplastyczny | = en |
| cs | Anaplastický oligodendrogliom | = en |
| uk | Анапластична олігодендрогліома | |
| zh | 间变性少突胶质细胞瘤 | |
| tr | Anaplastik oligodendrogliom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 137800 | NTBT | validated | |
| ICD-10 | C71.9 | NTBT | index | validated |
| ICD-11 | 2A00.0Y | NTBT | index | validated |
| ICD-11 | XH9QF3 | BTNT | specific | validated |
| MONDO | 0016696 | E | validated | |
| UMLS | C0334590 | E | validated | |
| GARD | 9472 | E | validated | |
| MedDRA | 10026659 | 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:46484Oligodendroglial tumor
rare neurological diseases; rare neoplastic diseases
No children.
Genes
2 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 | 23681562[PMID]_19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID] |
| POT1 | Major susceptibility factor in | Assessed | HGNC:17284 | ENSG00000128513 | 606478 | 7q31.33 | protection of telomeres 1 | 25482530[PMID] |
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.09 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.11 / 100 000 | United States | Value and class | Validated | Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/251630
- This record as JSON: /data/d/251630.json