Disease · MONDO_0007039

NF2-related schwannomatosis

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. NF2-related schwannomatosis. Diseases, record MONDO_0007039. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0007039

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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: NF2-related schwannomatosis; Open Targets
Open Targets ID: MONDO_0007039; Open Targets
Other names: NF2, SWNV, bilateral acoustic neurofibromatosis, central neurofibromatosis, full NF2, full neurofibromatosis type 2, neurofibromatosis 2, neurofibromatosis type 2, neurofibromatosis type II, nonmosaic NF2-related schwannomatosis, nonmosaic neurofibromatosis type 2; Open Targets
Disease Ontology: DOID:0111252; Disease Ontology
NCI Thesaurus: C3274; NCI Thesaurus
Orphanet: ORPHA:637; Orphanet
Highest stage of its drugs: Phase 2, for 9 of its 9 drugs, from ClinicalTrials.gov; Open Targets

Drugs9

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
EVEROLIMUS	CHEMBL1908360	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
LAPATINIB	CHEMBL554	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
AXITINIB	CHEMBL1289926	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
BEVACIZUMAB	CHEMBL1201583	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
CRIZOTINIB	CHEMBL601719	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
EMVODODSTAT	CHEMBL4650336	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ENDOSTATIN	CHEMBL4297747		Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ICOTINIB	CHEMBL2087361	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
LUVOMETINIB	CHEMBL6068059	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets14

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
EGFR	Epidermal growth factor receptor	2	Epidermal growth factor receptor erbB1 inhibitor	Phase 2	https://conductscience.org/library/genes/1956	Drugs and targets
ALK	ALK tyrosine kinase receptor	1	ALK tyrosine kinase receptor inhibitor; EML4-ALK inhibitor; NPM/ALK (Nucleophosmin/ALK tyrosine kinase receptor) inhibitor	Phase 2	https://conductscience.org/library/genes/238	Drugs and targets
DHODH	Dihydroorotate dehydrogenase (quinone), mitochondrial	1	Dihydroorotate dehydrogenase inhibitor	Phase 2	https://conductscience.org/library/genes/1723	Drugs and targets
EML4	EML4-ALK	1	EML4-ALK inhibitor	Phase 2	https://conductscience.org/library/genes/27436	Drugs and targets
ERBB2	Receptor tyrosine-protein kinase erbB-2	1	Receptor protein-tyrosine kinase erbB-2 inhibitor	Phase 2	https://conductscience.org/library/genes/2064	Drugs and targets
FKBP1A	Peptidyl-prolyl cis-trans isomerase FKBP1A	1	FK506-binding protein 1A inhibitor	Phase 2	https://conductscience.org/library/genes/2280	Drugs and targets
FLT1	Vascular endothelial growth factor receptor	1	Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/2321	Drugs and targets
FLT4	Vascular endothelial growth factor receptor	1	Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/2324	Drugs and targets
KDR	Vascular endothelial growth factor receptor	1	Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3791	Drugs and targets
MET	Hepatocyte growth factor receptor	1	Hepatocyte growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/4233	Drugs and targets

1 to 10 of 14

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Trials12

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.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT00863122	Concentration and Activity of Lapatinib in Vestibular Schwannomas	Early phase 1	26	2018-11-26	Vestibular Schwannoma, NF2, Neurofibromatosis 2, Acoustic Neuroma, Auditory Tumor	LAPATINIB	Its record names the disease	ClinicalTrials.gov
NCT01767792	Phase 2 Study of Bevacizumab in Children and Young Adults With NF 2 and Progressive Vestibular Schwannomas	Phase 2	22	2020-05-27	Neurofibromatosis Type 2, Progressive Vestibular Schwannomas	BEVACIZUMAB	Its record names the disease	ClinicalTrials.gov
NCT00973739	Lapatinib Study for Children and Adults With Neurofibromatosis Type 2 (NF2) and NF2-Related Tumors	Phase 2	21	2016-03-22	Neurofibromatosis 2, Vestibular Schwannoma	LAPATINIB	Its record names the disease	ClinicalTrials.gov
NCT02129647	Study of Axitinib in Patients With Neurofibromatosis Type 2 and Progressive Vestibular Schwannomas	Phase 2	13	2021-12-01	Neurofibromatosis Type 2, Vestibular Schwannomas	AXITINIB	Its record names the disease	ClinicalTrials.gov
NCT04283669	Phase 2 Clinical Trial of Crizotinib for Children and Adults With Neurofibromatosis Type 2 and Progressive Vestibular Schwannomas	Phase 2	12	2026-03-13	Neurofibromatosis 2, Progressive Vestibular Schwannoma (VS)	CRIZOTINIB	Its record names the disease	ClinicalTrials.gov
NCT01419639	Phase II Study of Everolimus (RAD001) in Children and Adults With Neurofibromatosis Type 2	Phase 2	10	2015-08-07	Neurofibromatosis Type II	EVEROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT06553365	Phase II Study of FCN-159 in NF2 Nerve Sheath Tumors	Phase 2	30		Nerve Sheath Tumor	LUVOMETINIB	Its record names the disease	ClinicalTrials.gov
NCT02104323	Endostatin Study for Patients With Neurofibromatosis Type 2 (NF2) and NF2-Related Tumors	Phase 2	20		Vestibular Schwannoma, Neurofibromatosis Type 2	ENDOSTATIN	Its record names the disease	ClinicalTrials.gov
NCT00911248	PTC299 for Treatment of Neurofibromatosis Type 2	Phase 2	11		Neurofibromatosis 2	EMVODODSTAT	Its record names the disease	ClinicalTrials.gov
NCT01490476	Efficacy and Safety Study of RAD001 in the Growth of the Vestibular Schwannoma(s) in Neurofibromatosis 2 (NF2) Patients	Phase 2	10		Neurofibromatosis 2	EVEROLIMUS	Its record names the disease	ClinicalTrials.gov

1 to 10 of 12

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Comparisons in its trials1

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.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial
Lapatinib vs Control: Assess the Level of ErbB2 Phosphorylation in VS.	5 of 9	2 of 4	13	NCT00863122

Source Trial comparisons

Papers from its trials1

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
38372904	DERIVED	1

Source PubMed

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_0007039

GET https://conductscience.org/api/diseases/v1/MONDO_0007039/drugs

API reference For AI assistants