Benign schwannoma
Synonyms: Neurilemmoma · Neurilemoma · Peripheral fibroblastoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare benign peripheral nerve sheath tumor characterized by a usually encapsulated space-occupying lesion composed of differentiated neoplastic Schwann cells. It most commonly arises from peripheral nerves in the head and neck region and extensor aspects of the extremities, but also from spinal and cranial nerves, especially the vestibular nerve. The tumor may be asymptomatic or cause symptoms related to a mass effect. It grows slowly and only rarely undergoes malignant transformation.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Benign schwannoma | Neurilemmoma · Neurilemoma · Peripheral fibroblastoma |
| fr | Schwannome bénin | Fibroblastome périphérique · Neurilemmome · Neurilémome |
| de | Schwannom, benignes | Fibroblastoma, peripheres · Neurilemmom · Neurolemmom |
| es | Schwannoma benigno | Fibroblastoma periférico · Neurilemoma |
| it | Schwannoma benigno | Fibroblastoma periferico · Neurilemmoma · Neurilemoma |
| nl | Goedaardig schwannoom | Neurilemmoom · Neurilemoom · Perifeer fibroblastoom |
| pt | Schwannoma benigno | Fibroblastoma periférico · Neurilemoma · Neurolemoma |
| pl | Łagodny nerwiak osłonkowy | Fibroblastoma obwodowa · Neurilemmoma · Neurilemoma · Neurolemmoma |
| cs | Benigní schwannom | Neurilemom · Periferní fibroblastom |
| uk | Доброякісна шваннома | Нейрилеммома · Нейрилема · Периферична фібробластома |
| zh | 良性神经鞘瘤 | 外周成纤维细胞瘤 |
| tr | Selim schwannom | Nörilemoma · Nörilemmoma · Periferik fibroblastom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D36.1 | NTBT | attributed | validated |
| ICD-11 | 2F3Y | NTBT | index | validated |
| MeSH | D009442 | E | validated | |
| UMLS | C0027809 | E | validated | |
| GARD | 4767 | E | validated | |
| MedDRA | 10029235 | 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:252131Benign peripheral nerve sheath tumor
rare neurological diseases; rare neoplastic diseases
Children (1):
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 6 / 100 000 | Europe | Value and class | Validated | European Medicines Agency[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
25 annotations · validated 2016-05-23| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001291 | Abnormal cranial nerve morphology | |
| HP:0009911 | Abnormal temporal bone morphology | |
| HP:0030177 | Abnormality of peripheral nervous system electrophysiology | |
| HP:0009593 | Peripheral schwannoma | |
| HP:0100008 | Schwannoma | |
| HP:0100011 | Scleral schwannoma | |
| HP:0009588 | Vestibular schwannoma | |
| Frequent (79-30%) | ||
| HP:0012533 | Allodynia | |
| HP:0010628 | Facial palsy | |
| HP:0000364 | Hearing abnormality | |
| HP:0002321 | Vertigo | |
| Occasional (29-5%) | ||
| HP:0002031 | Abnormal esophagus morphology | |
| HP:0002991 | Abnormal fibula morphology | |
| HP:0000197 | Abnormal parotid gland morphology | |
| HP:0000834 | Abnormality of the adrenal glands | |
| HP:0000769 | Abnormality of the breast | |
| HP:0001600 | Abnormality of the larynx | |
| HP:0001392 | Abnormality of the liver | |
| HP:0010826 | Abnormality of the twelfth cranial nerve | |
| HP:0003489 | Acute episodes of neuropathic symptoms | |
| HP:0200008 | Intestinal polyposis | |
| HP:0002011 | Morphological central nervous system abnormality | |
| HP:0100582 | Nasal polyposis | |
| HP:0012531 | Pain | |
| Excluded (0%) | ||
| HP:0100697 | Neurofibrosarcoma | |
Run the differential on the 7 obligate / very frequent terms
Annotation source: 8283540[PMID]_9326316[PMID]_8889506[PMID]_2577271[PMID]_25965126[PMID]_25549149[PMID]_25077322[PMID]_24978928[PMID]_23808475[PMID]_23681039[PMID]_16823047[PMID]_12437849[PMID]_8730376[PMID]_8578360[PMID]_7484619[PMID]_8427574[PMID]_2013780[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/252164
- This record as JSON: /data/d/252164.json