Ependymal tumor
- Type
- Clinical group
- Group
- Group of disorders
- Age of onset
- All ages
- Status
- active
A tumor of neurectodermal origin arising from ependymal cells that line the ventricles and central canal of the spinal cord, that can occur in both children and adults, and that is characterized by wide a range of clinical manifestations depending on the location of the tumor, such as intracranial hypertension for tumors originating in the posterior fossa, behavioural changes and pyramidal signs for supratentorial tumors, and dysesthesia for tumors of the spinal cord. They can be classified as myxopapillary ependymoma, subependymoma, ependymoma (low grade tumors) or anaplastic ependymoma (grade III tumors).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Ependymal tumor | |
| fr | Tumeur épendymaire | |
| de | Tumor, ependymaler | |
| es | Tumor ependimario | |
| it | Tumore ependimale | |
| nl | Ependymale tumor | |
| pt | Ependimoma | |
| pl | Guz pochodzenia ependymalnego | |
| cs | Ependymální nádor | |
| uk | Епендимальна пухлина | |
| zh | 室管膜肿瘤 | |
| tr | Ependimal tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 137800 | NTBT | validated | |
| MONDO | 0003266 | E | validated | |
| MONDO | 3266 | E | validated | |
| UMLS | C1333407 | 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:182067Glial tumor
rare neurological diseases; rare neoplastic diseases
Codes that Orphanet moved or referred to this one:
- ORPHA:251633OBSOLETE: Low-grade ependymoma referred to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
5 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.2 / 100 000 | Europe | Value and class | Validated | 22227039[PMID]_22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Lifetime Prevalence | 1-9 / 100 000 | 3.85 / 100 000 | Europe | Value and class | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.2 / 100 000 | United Kingdom | Value and class | Validated | 22227039[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.43 / 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/301
- This record as JSON: /data/d/301.json