Intraneural perineurioma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
Intraneural perineurioma is a rare tumor of cranial and spinal nerves arising from peripheral nerve sheath and composed exclusively or predominantly of cells showing perineurial differentiation. It presents as a localized, tubular or fusiform enlargement of a nerve or nerve segment, usually in the extremities or the trunk, associated with a motor-predominant mononeuropathy including slow, painless, gradual loss of motor function in the involved nerve trunk with muscle weakness and atrophy and, rarely, sensory dysfunction. Cranial nerve involvement is rare.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Intraneural perineurioma | |
| fr | Périneuriome intraneural | |
| de | Perineuriom, intraneurales | |
| es | Perineurioma intraneural | |
| it | Perineurinoma intraneurale | |
| nl | Intraneuraal perineurioom | |
| pt | Perineurioma intraneural | |
| pl | Guz okołonerwowy / Onerwiak śródnerwowy | |
| cs | Intraneurální perineuriom | |
| uk | Інтраневральна периневріома | |
| zh | 神经内神经鞘瘤 | |
| tr | İntranöral perinörom |
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 |
| ICD-11 | XH4BQ8 | E | specific | validated |
| MONDO | 0015032 | E | validated | |
| UMLS | C1370658 | E | validated | |
| GARD | 10921 | 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
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 | 172 cases | Worldwide | Case(s) | Validated | 34284162[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 34284162[PMID]_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/100003
- This record as JSON: /data/d/100003.json