Disease · MONDO_0017827

malignant peripheral nerve sheath tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. malignant peripheral nerve sheath tumor. Diseases, record MONDO_0017827. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0017827

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: malignant peripheral nerve sheath tumor; Open Targets
Open Targets ID: MONDO_0017827; Open Targets
Other names: Epithelioid MPNST, MPNST, MPNST - Malignant peripheral nerve sheath tumor, MPNST - Malignant peripheral nerve sheath tumour, MPNST with glandular differentiation, MPNST with mesenchymal differentiation, MPNSTs, Malig. periph. nerve sheath tum., Malignant Peripheral Nerve Sheath Tumors, Malignant Peripheral Nerve Sheath Tumour, Melanotic MPNST, Melanotic psammomatous MPNST, Neurofibrosarcoma, Neurofibrosarcoma [obs], Neurogenic sarcoma [obs], Neurosarcoma, Neurosarcoma [obs], Perineurioma, Perineuriomas, Peripheral Nerve Sheath Tumors, Malignant, malignant neoplasm of peripheral nerve sheath, malignant neoplasm of the peripheral nerve Sheath, malignant neurilemmoma, malignant neurilemoma, malignant neurofibroma, malignant peripheral nerve sheath neoplasm, malignant peripheral nerve sheath tumor (disorder), malignant peripheral nerve sheath tumor (morphologic abnormality), malignant peripheral nerve sheath tumor [dup] (morphologic abnormality), malignant peripheral nerve sheath tumour (morphologic abnormality), malignant peripheral nerve sheath tumour [dup] (morphologic abnormality), malignant schwannoma, malignant tumor of peripheral nerve sheath, malignant tumor of the peripheral nerve sheath, malignant tumour of peripheral nerve sheath, malignant tumour of the peripheral nerve sheath, neurofibrosarcoma, malignant, neurogenic sarcoma, schwannoma, malignant; Open Targets
Disease Ontology: DOID:5940; Disease Ontology
NCI Thesaurus: C3798; NCI Thesaurus
Orphanet: ORPHA:3148; Orphanet
Highest stage of its drugs: Phase 3, for 7 of its 39 drugs, from ClinicalTrials.gov; Open Targets

Drugs39

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
GEMCITABINE	CHEMBL888	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
IFOSFAMIDE	CHEMBL1024	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
DACARBAZINE	CHEMBL476	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
DOCETAXEL	CHEMBL3545252	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
EPIRUBICIN	CHEMBL417	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
TRABECTEDIN	CHEMBL450449	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
IMATINIB MESYLATE	CHEMBL1642	Small molecule	Phase 2/3	ClinicalTrials.gov	2	2	2	Drugs and targets
SELINEXOR	CHEMBL3545185	Small molecule	Phase 2	ClinicalTrials.gov	4	4	4	Drugs and targets
SIROLIMUS	CHEMBL413	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets

1 to 10 of 39

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Targets107

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
TUBA1A	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7846	Drugs and targets
TUBA1B	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/10376	Drugs and targets
TUBA1C	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/84790	Drugs and targets
TUBA3C	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7278	Drugs and targets
TUBA3E	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/112714	Drugs and targets
TUBA4A	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7277	Drugs and targets
TUBB	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/203068	Drugs and targets
TUBB1	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/81027	Drugs and targets
TUBB2A	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7280	Drugs and targets
TUBB2B	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/347733	Drugs and targets

1 to 10 of 107

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Trials30

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs. 1 more is not shown, since the checks do not confirm it.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT01653028	Alisertib in Treating Patients With Advanced or Metastatic Sarcoma	Phase 2	72	2016-12-28	Myxofibrosarcoma, Recurrent Adult Soft Tissue Sarcoma, Recurrent Leiomyosarcoma, Recurrent Liposarcoma, Recurrent Malignant Peripheral Nerve Sheath Tumor, Recurrent Undifferentiated Pleomorphic Sarcoma, Stage III Soft Tissue Sarcoma AJCC v7, Stage IV Soft Tissue Sarcoma AJCC v7	ALISERTIB	Its record names the disease	ClinicalTrials.gov
NCT02452554	Lorvotuzumab Mertansine in Treating Younger Patients With Relapsed or Refractory Wilms Tumor, Rhabdomyosarcoma, Neuroblastoma, Pleuropulmonary Blastoma, Malignant Peripheral Nerve Sheath Tumor, or Synovial Sarcoma	Phase 2	62	2019-08-29	Pleuropulmonary Blastoma, Recurrent Malignant Peripheral Nerve Sheath Tumor, Recurrent Neuroblastoma, Recurrent Rhabdomyosarcoma, Recurrent Synovial Sarcoma, Wilms Tumor	LORVOTUZUMAB MERTANSINE	Its record names the disease	ClinicalTrials.gov
NCT03872427	Testing Whether Cancers With Specific Mutations Respond Better to Glutaminase Inhibitor, Telaglenastat Hydrochloride, Anti-Cancer Treatment, BeGIN Study	Phase 2	54	2025-07-01	Advanced Malignant Solid Neoplasm, Metastatic Malignant Solid Neoplasm, NF1 Mutation Positive Malignant Peripheral Nerve Sheath Tumor, Unresectable Malignant Solid Neoplasm	TELAGLENASTAT	Its record names the disease	ClinicalTrials.gov
NCT00304083	Combination Chemotherapy in Treating Patients With Stage III or Stage IV Malignant Peripheral Nerve Sheath Tumors	Phase 2	48	2018-05-25	Neurofibromatosis Type 1, Sarcoma	DOXORUBICIN HYDROCHLORIDE, ETOPOSIDE, IFOSFAMIDE	Its record names the disease	ClinicalTrials.gov
NCT02584647	PLX3397 Plus Sirolimus in Unresectable Sarcoma and Malignant Peripheral Nerve Sheath Tumors	Phase 1/2	39	2025-03-21	Sarcoma, Malignant Peripheral Nerve Sheath Tumors	PEXIDARTINIB, SIROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT01661283	SARC016: Study of Everolimus With Bevacizumab to Treat Refractory Malignant Peripheral Nerve Sheath Tumors	Phase 2	25	2019-03-06	Malignant Peripheral Nerve Sheath Tumors, MPNST, Sarcoma	BEVACIZUMAB, EVEROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT00068367	S0330 Erlotinib in Treating Patients With Unresectable or Metastatic Malignant Peripheral Nerve Sheath Tumor	Phase 2	24	2017-11-06	Sarcoma	ERLOTINIB HYDROCHLORIDE	Its record names the disease	ClinicalTrials.gov
NCT02008877	SARC023: Ganetespib and Sirolimus in Patients With MPNST (Malignant Peripheral Nerve Sheath Tumors)	Phase 1/2	20	2019-05-02	Malignant Peripheral Nerve Sheath Tumors (MPNST), Sarcoma	GANETESPIB, SIROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT04917042	Tazemetostat in Malignant Peripheral Nerve Sheath Tumors	Phase 2	10	2026-02-04	Peripheral Nerve Sheath Tumor	TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT01710176	Localized High-Risk Soft Tissue Sarcomas Of The Extremities And Trunk Wall In Adults: An Integrating Approach Comprising Standard Vs Histotype-Tailored Neoadjuvant Chemotherapy	Phase 3	550		Localized High-risk Soft Tissue Sarcomas of the Extremities and Trunk Wall in Adults	DACARBAZINE, DOCETAXEL, EPIRUBICIN, ETOPOSIDE, GEMCITABINE, IFOSFAMIDE, TRABECTEDIN	Its record names the disease	ClinicalTrials.gov

1 to 10 of 30

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Comparisons in its trials0

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

None in this release.

Papers from its trials12

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
19451433	RESULT	1	PubMed
40595495	DERIVED	1	PubMed
39416174	DERIVED	1	PubMed
38232337	DERIVED	1	PubMed
34482181	DERIVED	1	PubMed
34321280	DERIVED	1	PubMed
32421444	DERIVED	1	PubMed
32089640	DERIVED	1	PubMed
28499583	DERIVED	1	PubMed
27733375	DERIVED	1	PubMed

1 to 10 of 12

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Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0017827

GET https://conductscience.org/api/diseases/v1/MONDO_0017827/drugs?page=2

API reference For AI assistants