Epithelioid malignant peripheral nerve sheath tumor

Summary

Epithelioid malignant peripheral nerve sheath tumor (MONDO:0004540) is a cancer and 2 clinical trials. Top therapeutic interventions include pazopanib, ifosfamide, and tazemetostat. A subtype of malignant peripheral nerve sheath 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	epithelioid malignant peripheral nerve sheath tumor
Mondo ID	MONDO:0004540
DOID	DOID:8353
NCIT	C6561
SNOMED CT	404038007
UMLS	C1321427
MedGen	272104
GARD	0024055
Is cancer (heuristic)	yes

Also known as: epithelioid malignant peripheral nerve sheath tumor · epithelioid MPNST · malignant epithelioid neoplasm of peripheral nerve sheath · malignant epithelioid neoplasm of the peripheral nerve sheath · malignant epithelioid peripheral nerve sheath neoplasm · malignant epithelioid peripheral nerve sheath tumor · malignant epithelioid peripheral nerve sheath tumour · malignant epithelioid tumor of peripheral nerve sheath · malignant epithelioid tumor of the peripheral nerve sheath · malignant epithelioid tumour of peripheral nerve sheath · malignant epithelioid tumour of the peripheral nerve sheath

Disease family

This is a subtype of malignant peripheral nerve sheath 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: 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 sarcoma › malignant peripheral nerve sheath tumor › epithelioid malignant peripheral nerve sheath tumor

Related subtypes (6): malignant cardiac peripheral nerve sheath neoplasm, malignant melanocytic neoplasm of the peripheral nerve sheath, childhood malignant schwannoma, adult malignant schwannoma, malignant glandular tumor of peripheral nerve sheath, malignant triton 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
PHASE2/PHASE3	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02180867	PHASE2/PHASE3	ACTIVE_NOT_RECRUITING	Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery
NCT03874455	Not specified	NO_LONGER_AVAILABLE	Tazemetostat Expanded Access Program for Adults With Solid Tumors

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PAZOPANIB	4	3
IFOSFAMIDE	4	1
TAZEMETOSTAT	4	1
CHEMBL4068768	0	1
CHEMBL4171277	0	1
CHEMBL5398431	0	1