Oligodendroglioma grade 2
Synonyms: Oligodendroglioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare glial tumor characterized by a highly cellular lesion that is diffusly infiltrating at the periphery and consists of evenly-spaced monomorphic cells with the oligodendroglial phenotype. It typically occurs in the supratentorial white matter. Histologically, the cells are uniformly round to oval with round nuclei, delicate chromatin and small nucleoli. Most patients present with seizures.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Oligodendroglioma grade 2 | Oligodendroglioma |
| fr | Oligodendrogliome grade 2 | Oligodendrogliome |
| de | Oligodendrogliom Grad 2 | Oligodendrogliom |
| es | Oligodendroglioma de grado 2 | = en |
| it | Oligodendroglioma | |
| nl | Oligodendroglioom graad 2 | Oligodendroglioom |
| pt | Oligodendroglioma | |
| pl | Skąpodrzewiak (Oligodendroglioma) | = en |
| cs | Oligodendrogliom | = en |
| uk | Олігодендрогліома | |
| zh | 少突胶质细胞瘤 | |
| tr | 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 | XH7K31 | BTNT | specific | validated |
| ICD-11 | XH7W59 | BTNT | specific | validated |
| MONDO | 0016695 | E | validated | |
| MeSH | D009837 | E | validated | |
| UMLS | C0028945 | E | validated | |
| GARD | 9953 | E | validated | |
| MedDRA | 10030286 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.25 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
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/251627
- This record as JSON: /data/d/251627.json