Extraneural perineurioma
Synonyms: Soft tissue perineurioma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly, Infancy
- Status
- active
Extraneural perineurioma is a rare tumor of cranial and spinal nerves arising from peripheral nerve sheet and composed exclusively or predominantly of cells showing perineurial differentiation. It presents as a well-circumscribed, rarely encapsulated mass, not associated with a recognizable nerve, most commonly arising in the dermis and subcutis of the extremities or trunk, or, rarely, in deep soft tissue or skin (e.g., in the stomach, kidney, pancreas, maxillary sinus, mandible, bronchial tree and the face). The clinical presentation depends on the localization.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Extraneural perineurioma | Soft tissue perineurioma |
| fr | Périneuriome extraneural | Périneuriome des tissus mous |
| de | Perineuriom, extraneurales | Weichteilperineuriom |
| es | Perineurioma extraneural | Perineurioma de tejidos blandos |
| it | Perineurinoma extraneurale | Perineurinoma dei tessuti molli |
| nl | Extraneuraal perineurioom | Perineurioom van de weke delen |
| pt | Perineurioma extraneural | |
| pl | Nerwiak zewnątrznerwowy | Guz okołonerwowy/Onerwiak tkanek miękkich |
| cs | Extraneurální perineuriom | Perineuriom z měkkých tkání |
| uk | Екстраневральна периневріома | Периневрома мяких тканин |
| zh | 神经外神经鞘瘤 | 软组织神经膜瘤 |
| tr | Ekstranöral perinörom | Yumuşak doku perinöryomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D36.1 | BTNT | attributed | validated |
| ICD-11 | 2F3Y | NTBT | index | validated |
| MONDO | 0015031 | E | validated | |
| UMLS | C4708595 | 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:85102Perineurioma
rare neurological diseases; rare neoplastic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 300 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100002
- This record as JSON: /data/d/100002.json