Nerve sheath neoplasm

Summary

Nerve sheath neoplasm (MONDO:0002547) is a cancer (an umbrella term covering 6 Mondo subtypes) and 6 clinical trials. Top therapeutic interventions include pazopanib, ifosfamide, and luvometinib. A subtype of peripheral nervous system neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Umbrella term: 6 Mondo subtypes
• Clinical trials: 6

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	nerve sheath neoplasm
Mondo ID	MONDO:0002547
MeSH	D018317
DOID	DOID:3193
NCIT	C4972
UMLS	C0206727
MedGen	64639
GARD	0023161
Is cancer (heuristic)	yes

Also known as: neoplasm of nerve sheath · neoplasm of the nerve sheath · nerve sheath neoplasm · nerve sheath tumor · tumor of nerve sheath · tumour of nerve sheath

Disease family

This is a subtype of peripheral nervous system neoplasm. 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 › nerve sheath neoplasm

Related subtypes (8): autonomic nervous system 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

Subtypes (6): schwannoma, granular cell tumor, neurothekeoma, neurofibroma, malignant peripheral nerve sheath tumor, perineurioma

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: 6.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	4
PHASE2/PHASE3	1
PHASE2	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
NCT06553365	PHASE2	RECRUITING	Phase II Study of FCN-159 in NF2 Nerve Sheath Tumors
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)
NCT06041347	Not specified	UNKNOWN	Learning Curve for the Visualization of Sacral Plexus on TVS

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PAZOPANIB	4	3
IFOSFAMIDE	4	1
LUVOMETINIB	2	1
CHEMBL4068768	0	1
CHEMBL4171277	0	1