Central nervous system primitive neuroectodermal neoplasm
diseaseOn this page
Also known as central nervous system neuroectodermal tumorcentral nervous system neuroectodermal tumourcentral nervous system PNETcentral nervous system primitive neuroectodermal tumorcentral nervous system primitive neuroectodermal tumor (WHO grade IV)central nervous system primitive neuroectodermal tumourcentral nervous system primitive neuroectodermal tumour (WHO grade IV)central primitive neuroectodermal neoplasmcentral primitive neuroectodermal tumorcentral primitive neuroectodermal tumourCNS PNETCNS primitive neuroectodermal neoplasmCNS primitive neuroectodermal tumorCNS primitive neuroectodermal tumourprimitive neuroectodermal tumor of central nervous systemprimitive neuroectodermal tumour of central nervous system
Summary
Central nervous system primitive neuroectodermal neoplasm (MONDO:0000640) is a cancer (an umbrella term covering 8 Mondo subtypes). A subtype of central nervous system cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Umbrella term: 8 Mondo subtypes
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | central nervous system primitive neuroectodermal neoplasm |
| Mondo ID | MONDO:0000640 |
| DOID | DOID:0060103 |
| ICD-11 | 1711526170 |
| NCIT | C5398 |
| UMLS | C3887678 |
| MedGen | 854517 |
| GARD | 0027536 |
| Anatomy (UBERON) | UBERON:0001017 |
| Is cancer (heuristic) | yes |
Also known as: central nervous system neuroectodermal tumor · central nervous system neuroectodermal tumour · central nervous system PNET · central nervous system primitive neuroectodermal neoplasm · central nervous system primitive neuroectodermal tumor · central nervous system primitive neuroectodermal tumor (WHO grade IV) · central nervous system primitive neuroectodermal tumour · central nervous system primitive neuroectodermal tumour (WHO grade IV) · central primitive neuroectodermal neoplasm · central primitive neuroectodermal tumor · central primitive neuroectodermal tumour · CNS PNET · CNS primitive neuroectodermal neoplasm · CNS primitive neuroectodermal tumor · CNS primitive neuroectodermal tumour · primitive neuroectodermal tumor of central nervous system · primitive neuroectodermal tumour of central nervous system
Disease family
This is a subtype of central nervous system cancer. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › nervous system cancer › central nervous system cancer › central nervous system primitive neuroectodermal neoplasm
Related subtypes (19): brain cancer, central nervous system sarcoma, primary central nervous system lymphoma, central nervous system germinoma, central nervous system melanocytic neoplasm, central nervous system endodermal sinus tumor, spinal cord cancer, malignant carotid body paraganglioma, malignant adrenal gland pheochromocytoma, malignant jugulotympanic paraganglioma, pheochromocytoma/paraganglioma syndrome 2, pheochromocytoma/paraganglioma syndrome 5, central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor, choriocarcinoma of the central nervous system, mixed germ cell tumor of central nervous system, embryonal carcinoma of the central nervous system, malignant tumor of meninges, malignant central nervous system mesenchymal, non-meningothelial neoplasm, malignant glioma
Subtypes (8): adult central nervous system primitive neuroectodermal neoplasm, childhood central nervous system primitive neuroectodermal neoplasm, intracranial primitive neuroectodermal tumor, spinal cord neuroblastoma, ganglioneuroma, spinal cord primitive neuroectodermal tumor, ependymoblastoma, central nervous system tumor with bcor internal tandem duplication
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 0.
Related Atlas pages
No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.