Central neurocytoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
Central neurocytoma is a very rare brain tumor of young adults (over 100 cases reported worldwide). It is typically found in the lateral ventricles and occasionally in the third ventricle. Symptoms are those of increased intracranial pressure: headache, nausea and vomiting, drowsiness, vision problems and mental changes. Total removal of the tumor is the therapy of choice. Post-operative prognosis is generally good.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Central neurocytoma | |
| fr | Neurocytome central | |
| de | Neurozytom, zentrales | |
| es | Neurocitoma central | |
| it | Neurocitoma centrale | |
| nl | Centraal neurocytoom | |
| pt | Neurocitoma central | |
| pl | Neurocytoma ośrodkowa | |
| cs | Centrální neurocytom | |
| uk | Центральна нейроцитома | |
| zh | 中枢神经细胞瘤 | |
| tr | Santral nörositoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.0 | NTBT | attributed | validated |
| ICD-11 | 2A00.3 | E | specific | validated |
| MONDO | 0019134 | E | validated | |
| UMLS | C0206719 | E | validated | |
| GARD | 10641 | 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:251924Neuronal tumor
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 | 500 cases | Worldwide | Case(s) | Validated | 23810386[PMID] | |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 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
19 annotations · validated 2016-08-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0000238 | Hydrocephalus | |
| Very frequent (99-80%) | ||
| HP:0025354 | Abnormal cellular phenotype | |
| HP:0030047 | Abnormality of lateral ventricle | |
| HP:0010576 | Intracranial cystic lesion | |
| Frequent (79-30%) | ||
| HP:0000504 | Abnormality of vision | |
| HP:0001251 | Ataxia | |
| HP:0002514 | Cerebral calcification | |
| HP:0008000 | Decreased corneal reflex | |
| HP:0000716 | Depression | |
| HP:0002315 | Headache | |
| HP:0002516 | Increased intracranial pressure | |
| HP:0002017 | Nausea and vomiting | |
| Occasional (29-5%) | ||
| HP:0003487 | Babinski sign | |
| HP:0001259 | Coma | |
| HP:0001254 | Lethargy | |
| HP:0007021 | Pain insensitivity | |
| HP:0003401 | Paresthesia | |
| HP:0002172 | Postural instability | |
| HP:0000360 | Tinnitus | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 3776470[PMID]_15015671[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/73256
- This record as JSON: /data/d/73256.json