central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor

Summary

central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor (MONDO:0016713) is a cancer. 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
• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.07	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor
Mondo ID	MONDO:0016713
Orphanet	251870
NCIT	C129537
UMLS	C4329632
MedGen	1389430
GARD	0020718
MedDRA	10057846
Anatomy (UBERON)	UBERON:0001017
Is cancer (heuristic)	yes

Also known as: central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor · central nervous system PNET · central nervous system primitive neuroectodermal tumour

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 Ewing sarcoma/peripheral primitive neuroectodermal tumor

Related subtypes (19): central nervous system primitive neuroectodermal neoplasm, 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, 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 (4): medulloepithelioma, esthesioneuroblastoma, ependymoblastoma, embryonal tumor with multilayered rosettes

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.