Vestibular schwannoma
Synonyms: Acoustic neurilemoma · Acoustic neurinoma · Acoustic neuroma
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Status
- active
Vestibular schwannoma is a rare tumor of the posterior fossa originating in the Schwann cells of the vestibular transitional zone of the vestibulocochlear nerve that can be benign, small, slow growing and asymptomatic or large, faster growing and aggressive and potentially fatal, presenting with symptoms of hearing and balance impairment, vertigo, ataxia, headache and fifth, sixth or seventh cranial nerve dysfunction and facial numbness.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vestibular schwannoma | Acoustic neurilemoma · Acoustic neurinoma · Acoustic neuroma |
| fr | Neurinome de l'acoustique | Schwannome vestibulaire |
| de | Schwannom, vestibuläres | Akustikusneurinom · Vestibularis-Schwannom |
| es | Schwannoma vestibular | Neurilemoma acústico · Neurinoma acústico · Neuroma acústico |
| it | Schwannoma vestibolare | Neurilemmoma del nervo acustico · Neurinoma del nervo acustico · Neuroma del nervo acustico |
| nl | Vestibulair schwannoom | Akoestisch neurilemoom · Akoestisch neurinoom · Akoestisch neuroom |
| pt | Schwannoma vestibular | Neurilemoma do acústico · Neurinoma do acústico · Neuroma do acústico |
| pl | Nerwiak osłonkowy przedsionkowy | Neurilemmoma nerwu słuchowego · Nerwiak nerwu słuchowego |
| cs | Vestibulární schwannom | Neurilemom akustiku · Neurinom akustiku · Neurom akustiku |
| uk | Вестибулярна шваннома | Акустична невринома · Акустична невринома · Акустична неврома |
| zh | 前庭神经鞘瘤 | 听神经鞘瘤 |
| tr | Vestibüler schwannom | Akustik nöroma · Akustik nörinom · Akustik nörilemoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.3 | NTBT | index | validated |
| ICD-11 | 2A02.3 | NTBT | index | validated |
| MONDO | 0001569 | E | validated | |
| MeSH | D009464 | E | validated | |
| UMLS | C0027859 | E | validated | |
| GARD | 223 | E | validated | |
| MedDRA | 10000523 | 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 (2):
- ORPHA:252164Benign schwannoma
rare neurological diseases; rare neoplastic diseases - ORPHA:98061Rare otorhinolaryngologic tumor
rare otorhinolaryngological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 2 / 100 000 | Denmark | Value and class | Validated | 20871439[PMID]_24655069[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.2 / 100 000 | United States | Value and class | Validated | National Cancer OTHERitute[OTHER]_23432451[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.8 / 100 000 | Specific population | Value and class | Validated | 21897393[PMID] |
| Annual incidence | 1-9 / 100 000 | — | Europe | Class only | Not yet 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/252175
- This record as JSON: /data/d/252175.json