Dermoid or epidermoid cyst of the central nervous system
Synonyms: Dermoid or epidermoid cyst of the CNS
- Type
- Morphological anomaly
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare congenital tumor characterized by a benign cyst with epithelial and epidermoid components, originating from embryologic displacement and ectopic growth of ectodermal tissue in the central nervous system. In contrast to epidermoid cysts, dermoid cysts also contain dermis and skin appendages. Most common location is the lumbosacral region, as well as the cerebellopontine angle and parasellar area for intracranial lesions. Clinical presentation depends on the location and size of the tumor and includes pain, muscle weakness, motor and sensory disturbances, and incontinence for intraspinal lesions, and intracranial hypertension, gait disturbances, cranial nerve dysfunction, and visual deficits for intracranial tumors. The cysts may rupture and cause chemical meningitis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Dermoid or epidermoid cyst of the central nervous system | Dermoid or epidermoid cyst of the CNS |
| fr | Kyste dermoïde ou epidermoïde du système nerveux central | Kyste dermoïde ou epidermoïde du SNC |
| de | Dermoid oder Dermoidzyste des Zentralnervensystems | Dermoid oder Dermoidzyste des ZNS |
| es | Quiste dermoide o epidermoide del sistema nervioso central | Quiste dermoide o epidermoide del SNC |
| it | Cisti dermoide o epidermoide del sistema nervoso centrale | |
| nl | Dermoïd- of epidermoïdcyste van centraal zenuwstelsel | Dermoïd- of epidermoïdcyste van CZS |
| pt | Quisto dermóide ou epidermóide do sistema nervoso central | |
| pl | Torbiel skórzasta lub epidermoidalna ośrodkowego układu nerwowego | = en |
| cs | Dermoidní nebo epidermoidní cysta centrálního nervového systému | Dermoidní nebo epidermoidní cysta CNS |
| uk | Дермоїдна або епідермоїдна кіста центральної нервової системи | Дермоїдна або епідермоїдна кіста ЦНС |
| zh | 中枢神经系统的皮样或表皮样囊肿 | = en |
| tr | Merkezi sinir sisteminde dermoid veya epidermoid kisti | CNS'nin dermoid veya epidermoid kisti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q07.8 | NTBT | attributed | not yet validated |
| MONDO | 0035159 | E | validated | |
| UMLS | C5680190 | 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:269194Central nervous system cystic malformation
rare developmental anomalies during embryogenesis; rare neurological diseases - ORPHA:730707Genetic forebrain malformation
rare genetic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/530033
- This record as JSON: /data/d/530033.json