raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:251927

Extraventricular neurocytoma

Synonyms: EVN

Type
Disease
Group
Disorder
Status
active

Extraventricular neurocytoma (EVN), a variant of central neurocytoma, is a rare neuronal neoplasm, composed of round cells with neuronal differentiation, which is located outside of the ventricular system, usually within the spinal cord or cerebral hemispheres and that manifests with headache, nausea, vomiting, complex partial seizures or focal neurological deficits. In some cases it may exhibit atypical features consistent with aggressive clinical behavior.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enExtraventricular neurocytomaEVN
frNeurocytome extraventriculaire= en
deNeurozytom, extraventrikuläres= en
esNeurocitoma extraventricular= en
itNeurocitoma extraventricolare
nlExtraventriculair neurocytoom= en
ptNeurocitoma extraventricular
plNerwiak komórkowy pozakomorowy= en
csExtraventrikulární neurocytom= en
ukПозашлуночкова нейроцитома= en
zh脑室外神经细胞瘤= en
trEkstraventriküler nörositom= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C72.9NTBTattributedvalidated
ICD-112A00.3NTBTattributedvalidated
ICD-11XH2HS1Especificvalidated
MONDO0016727Evalidated
UMLSC2985175Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card