Peripheral primitive neuroectodermal tumor of soft tissues

Summary

Peripheral primitive neuroectodermal tumor of soft tissues (MONDO:0002982) is a cancer and 2 clinical trials. Top therapeutic interventions include dexrazoxane, ifosfamide, and vincristine. A subtype of peripheral primitive neuroectodermal tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Clinical trials: 2

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	peripheral primitive neuroectodermal tumor of soft tissues
Mondo ID	MONDO:0002982
DOID	DOID:4389
NCIT	C27471
UMLS	C1112437
MedGen	205115
GARD	0023313
Is cancer (heuristic)	yes

Also known as: peripheral neuroectodermal tumor of soft tissues · peripheral neuroectodermal tumor of the soft tissues · peripheral neuroectodermal tumour of soft tissues · peripheral neuroectodermal tumour of the soft tissues · peripheral neuroepithelioma of soft tissues · peripheral neuroepithelioma of the soft tissues · peripheral primitive neuroectodermal tumor of soft tissues

Disease family

This is a subtype of peripheral primitive neuroectodermal tumor. 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 › embryonal neoplasm › primitive neuroectodermal tumor › peripheral primitive neuroectodermal tumor › peripheral primitive neuroectodermal tumor of soft tissues

Related subtypes (2): peripheral primitive neuroectodermal tumor of bone, Askin tumor

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: 2.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE3	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02306161	PHASE3	ACTIVE_NOT_RECRUITING	Combination Chemotherapy With or Without Ganitumab in Treating Patients With Newly Diagnosed Metastatic Ewing Sarcoma
NCT01231906	PHASE3	COMPLETED	Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DEXRAZOXANE	4	3
IFOSFAMIDE	4	2
VINCRISTINE	4	2
ETOPOSIDE PHOSPHATE	4	1
TOPOTECAN HYDROCHLORIDE	4	1
GANITUMAB	3	1