Peripheral nervous system neoplasm

Summary

Peripheral nervous system neoplasm (MONDO:0001406) is a cancer (an umbrella term covering 9 Mondo subtypes) and 8 clinical trials. Top therapeutic interventions include pazopanib, ifosfamide, and pelabresib. A subtype of peripheral nervous system disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Umbrella term: 9 Mondo subtypes
• Clinical trials: 8

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	peripheral nervous system neoplasm
Mondo ID	MONDO:0001406
EFO	EFO:0002431
MeSH	D010524
DOID	DOID:1192
NCIT	C3321
SNOMED CT	126980002
UMLS	C0031118
MedGen	45817
Anatomy (UBERON)	UBERON:0000010
Is cancer (heuristic)	yes

Also known as: CNS-excluded nervous sys. cancer · neoplasm of peripheral nerve · neoplasm of peripheral nervous system · neoplasm of PNS · neoplasm of the peripheral nerve · neoplasm of the peripheral nervous system · neoplasm of the PNS · neoplasms, peripheral nervous system · neoplasms, PNS · nerve sheath neoplasm · nerve sheath tumors · nerve sheath tumours · peripheral nerve neoplasm · peripheral nerve tumor · peripheral nerve tumour · peripheral nervous system neoplasm · peripheral nervous system neoplasm (disease) · peripheral nervous system tumor · peripheral nervous system tumour · PNS neoplasm (+15 more)

Disease family

This is a subtype of peripheral nervous system disorder. 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 body system or component › nervous system disorder › peripheral nervous system disorder › peripheral nervous system neoplasm

Related subtypes (17): autoimmune disorder of peripheral nervous system, autonomic nervous system disorder, vestibulocochlear nerve disorder, hypoglossal nerve disorder, facial nerve disorder, neuroma, accessory nerve disorder, glossopharyngeal nerve disorder, olfactory nerve disorder, radiculopathy, trigeminal nerve disorder, third cranial nerve disorder, peripheral neuropathy, cauda equina syndrome, peroneal nerve paralysis, trochlear nerve disorder, abducens nerve disorder

Subtypes (9): autonomic nervous system neoplasm, nerve sheath neoplasm, cranial nerve neoplasm, mediastinal neural neoplasm, nerve plexus neoplasm, nerve root neoplasm, peripheral ganglioneuroblastoma, peripheral nervous system cancer, benign neoplasm of peripheral nervous system

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

Drugs indicated or in trials for this disease

No drug has an approved disease-direct ChEMBL indication for this disease.

1 drug in clinical trials for this disease (phase 2–3, investigational): efficacy not established — a trial record, not an indication.

Drug	Highest phase
Pelabresib	Phase 2

Clinical trials & evidence

Clinical trials

Clinical trials: 8.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	5
PHASE2/PHASE3	1
PHASE2	1
PHASE1	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
NCT02986919	PHASE2	WITHDRAWN	Study of CPI-0610 in Patients With Malignant Peripheral Nerve Sheath Tumors
NCT04465643	PHASE1	COMPLETED	Neoadjuvant Nivolumab Plus Ipilimumab for Newly Diagnosed Malignant Peripheral Nerve Sheath Tumor
NCT04278118	Not specified	RECRUITING	Hypofractionated Proton Therapy for Benign Intracranial Brain Tumors, the HiPPI Study
NCT04648462	Not specified	RECRUITING	Proton Therapy Research Infrastructure- ProTRAIT- Neuro-oncology
NCT06222203	Not specified	RECRUITING	Surveillance for Malignant Transformation of Neurofibromatosis Type 1 (NF1) Related Peripheral Nerve Sheath Tumors (PNST)
NCT01567787	Not specified	WITHDRAWN	Proton Radiation Therapy for Spinal Tumors
NCT05735548	Not specified	UNKNOWN	Tumors of the Peripheral Nervous System

Drugs tested across these trials (top 30)

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