raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:251870

Central nervous system embryonal tumor

Synonyms: CNS PNET · Central nervous system primitive neuroectodermal tumor

Type
Clinical group
Group
Group of disorders
Inheritance
Not applicable
Age of onset
Childhood
Status
active

This term does not characterize a disease but a group of diseases. To learn about the diseases included under this term, you can consult the classifications.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCentral nervous system embryonal tumorCNS PNET · Central nervous system primitive neuroectodermal tumor
frTumeur embryonnaire du système nerveux centralTNEP du SNC · Tumeur neuroectodermique primitive du système nerveux central
deZNS-Tumor, embryonalerPNET des Zentralnervensystems · Primitiver neuroektodermaler Tumor des Zentralnervensystems · ZNS- PNET
esTumor embrionario del sistema nervioso centralCNS PNET · TNEP SNC · Tumor neuroectodérmico primitivo del sistema nervioso central
itTumore neuroectodermico primitivo del sistema nervoso centralePNET CNS · PNET del sistema nervoso centrale
nlEmbryonale tumor van centraal zenuwstelselCZS-PNET · Primitieve neuro-ectodermale tumor van centraal zenuwstelsel
ptTumor neuroectodérmico primitivoPNET
plNiezróżnicowany guz neuroektodermalny centralnego układu nerwowegoPNET centralnego układu nerwowego · PNET CUN
csPrimitivní neuroektodermální nádor CNSCNS PNET · PNET centrálního nervového systému
ukЕмбріональна пухлина центральної нервової системиПримітивна нейроектодермальна пухлина центральної нервової системи · CNS PNET
zh中枢神经系统胚胎瘤中枢神经系统原发性神经内分泌肿瘤
trMerkezi sinir sistemi embriyonal tümörüCNS PNET · Merkezi sinir sistemi primitif nöroektodermal tümör
2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO16713Evalidated
UMLSC1333378Evalidated

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):

Children (4):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.07 / 100 000EuropeValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]

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