Chordoid glioma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare central nervous system tumor characterized by a non-infiltrative and well-circumscribed WHO grade 2 glial neoplasm occurring in the third ventricle, often extending to hypothalamus. Chordoid glioma presents most frequently in middle-aged women with symptoms of memory deficits, headaches and visual loss, related to its location. Virtually all chordoid gliomas exhibit positivity for GFAP and TTF1 immunostaining, <i>PRKCA</i>p.D463H mutation, and a specific chordoid glioma DNA methylation class.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Chordoid glioma | |
| fr | Gliome chordoïde | |
| de | Chordoidgliom | |
| es | Glioma cordoide | |
| it | Glioma cordoide | |
| nl | Chordoïd glioom | |
| pt | Glioma cordóide | |
| pl | Glejak struniakowy | |
| cs | Chordoidní gliom | |
| uk | Хордоїдна гліома | |
| zh | 脊索样胶质瘤 | |
| tr | Kordoid gliom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C71.9 | NTBT | attributed | validated |
| ICD-11 | 2A00.0Y | NTBT | index | validated |
| ICD-11 | XH9HV1 | E | specific | validated |
| UMLS | C1322252 | 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:717268Circumscribed astrocytic glioma
rare neurological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 80 cases | Worldwide | Case(s) | Validated | 25648470[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 25648470[PMID]_ORPHANET |
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/251674
- This record as JSON: /data/d/251674.json