raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:251646

Anaplastic ependymoma

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Childhood
Status
active

A rare, malignant type of ependymoma that most often arises in the supratentorial region of the brain of children and young adults and that manifests with variable symptoms including headaches, nausea, vision impairment, memory loss and difficulty walking.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAnaplastic ependymoma
frEpendymome anaplasique
deEpendymom, anaplastisches
esEpendimoma anaplásico
itEpendimoma anaplasticoEpendimoma di alto grado
nlAnaplastisch ependymoom
ptEpendimona anaplásicoEpendimona de alto grau
plWyściółczak anaplastycznyWyściółczak o wysokim stopniu złośliwości
csAnaplastický ependymomHigh-grade ependymom
ukАнапластична епендимома
zh间变性室管膜瘤
trAnaplastik ependimom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C71.9NTBTindexvalidated
ICD-112A00.0YNTBTindexvalidated
ICD-11XH6922Especificvalidated
MONDO0016700Evalidated
UMLSC0280788Evalidated
GARD10634Evalidated
MedDRA10014968Evalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.04 / 100 000United StatesValue and classValidated23660944[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

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

7

Sources for this card