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malignant peripheral nerve sheath tumor - Clinical Profile, Genes & Research | Kisho | Kisho

No approved treatments are currently available for malignant peripheral nerve sheath tumor. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for malignant peripheral nerve sheath tumor, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for malignant peripheral nerve sheath tumor. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor	Designated	Exclusivity End	Designation Status
Small molecule inhibitor of PRMT5 (protein arginine methyl transferase 5)	Small molecule inhibitor of PRMT5 (protein arginine methyl transferase 5)	Tango Therapeutics	2022	—	Designated

Gene therapy approaches for malignant peripheral nerve sheath tumor have been reported in the published literature.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

27 clinical trials registered, 15 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 10 PHASE2, 10 PHASE1, 1 EARLY_PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT05985161](https://clinicaltrials.gov/study/NCT05985161)	A Study of Selinexor in People With Wilms Tumors and Other Solid Tumors	PHASE2	Memorial Sloan Kettering Cancer Center	RECRUITING
[NCT07298083](https://clinicaltrials.gov/study/NCT07298083)	DoD Award NF230020 Identification of Metabolic Markers and Statistical Prediction of MPNST for Rapid Diagnosis and Assessment of Surgical Margins	—	M.D. Anderson Cancer Center	RECRUITING
[NCT06849986](https://clinicaltrials.gov/study/NCT06849986)	IO Combined With AI as First-line Treatment for Patients With Soft Tissue Sarcoma(TAIS)	PHASE2	Fudan University	RECRUITING
[NCT04872543](https://clinicaltrials.gov/study/NCT04872543)	A Study of ASTX727 in People With Malignant Peripheral Nerve Sheath Tumors (MPNST)	PHASE2	Memorial Sloan Kettering Cancer Center	RECRUITING
[NCT04222413](https://clinicaltrials.gov/study/NCT04222413)	Metarrestin (ML-246) in Subjects With Metastatic Solid Tumors	PHASE1	National Cancer Institute (NCI)	RECRUITING

209 publications have been identified in PubMed for malignant peripheral nerve sheath tumor. Kisho has analyzed 102 by research type. Research spans Case Report / Case Series (42%), Review / Meta-Analysis (17%), and Basic Science / Preclinical (17%).

Research Type	Count	% of Total
Patient case studies	43	42%
Research summaries	17	17%
Laboratory research	17	17%
Testing and diagnosis research	11	11%
Disease patterns and progression	5	5%
Clinical study results	4

Recent Publications

Abdallah H (2026). [PMID: 41484915](https://pubmed.ncbi.nlm.nih.gov/41484915/). *J Cardiothorac Surg*. [Case Report / Case Series]
Porche K (2026). [PMID: 41237389](https://pubmed.ncbi.nlm.nih.gov/41237389/). *J Neurosurg*. [Case Report / Case Series]
Dry S (2026). [PMID: 41183597](https://pubmed.ncbi.nlm.nih.gov/41183597/). *Mod Pathol*. [Review / Meta-Analysis]
Gui C (2026). [PMID: 41769416](https://pubmed.ncbi.nlm.nih.gov/41769416/). *Neurooncol Adv*. [Review / Meta-Analysis]
Aubrey RE (2026). [PMID: 41568612](https://pubmed.ncbi.nlm.nih.gov/41568612/). *Cancer*. [Epidemiology / Natural History]
Ye R (2026). [PMID: 41521307](https://pubmed.ncbi.nlm.nih.gov/41521307/). *Neurol Sci*. [Case Report / Case Series]
Muramatsu S (2026). [PMID: 41073222](https://pubmed.ncbi.nlm.nih.gov/41073222/). *J Orthop Sci*. [Epidemiology / Natural History]
Qian L (2026). [PMID: 41526870](https://pubmed.ncbi.nlm.nih.gov/41526870/). *BMC Surg*. [Case Report / Case Series]
Aytaç A (2026). [PMID: 41377613](https://pubmed.ncbi.nlm.nih.gov/41377613/). *Radiol Case Rep*. [Case Report / Case Series]
Porche K (2026). [PMID: 41237393](https://pubmed.ncbi.nlm.nih.gov/41237393/). *Journal of neurosurgery*. [Basic Science / Preclinical]

Kisho

malignant peripheral nerve sheath tumor

Overview

Diagnosis

Treatment

Orphan Drug Designations

Clinical Trials

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Latest News & Developments

Molecular subgroups of human malignant peripheral nerve sheath tumors are conserved in canines.

Malignant Peripheral Nerve Sheath Tumour of the Adrenal Gland: An Unusual Pathology in an Adrenal Incidentaloma.

Cytokeratin-Positive Pleomorphic Chest Wall Sarcoma Mimicking MPNST: A Case Report.

malignant peripheral nerve sheath tumor

Overview

Diagnosis

Treatment

Orphan Drug Designations

Clinical Trials

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Latest News & Developments

Molecular subgroups of human malignant peripheral nerve sheath tumors are conserved in canines.

Malignant Peripheral Nerve Sheath Tumour of the Adrenal Gland: An Unusual Pathology in an Adrenal Incidentaloma.

Cytokeratin-Positive Pleomorphic Chest Wall Sarcoma Mimicking MPNST: A Case Report.