Disease · EFO_1001106
pigmented villonodular synovitis
6 sourcesdiseases-2026-09-18-0.1.0
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ConductScience Library. pigmented villonodular synovitis. Diseases, record EFO_1001106. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/EFO_1001106
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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.
- Other names
Diffuse Giant cell tumor of Tenosynovium, Synovitis, Pigmented Villonodular, TGCT, TSGCT, diffuse giant cell neoplasm of Tenosynovium, diffuse giant cell neoplasm of tendon sheath, diffuse giant cell neoplasm of the Tenosynovium, diffuse giant cell tumor of tendon sheath, diffuse giant cell tumor of the Tenosynovium, diffuse giant cell tumour of Tenosynovium, diffuse giant cell tumour of tendon sheath, diffuse giant cell tumour of the Tenosynovium, diffuse tenosynovial giant cell neoplasm, diffuse tenosynovial giant cell tumor, diffuse tenosynovial giant cell tumour, tenosynovial giant cell tumor, diffuse type, villous tenosynovitis
- Open Targets
Drugs13
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 |
|---|---|---|---|---|---|---|---|---|
| PEXIDARTINIB | CHEMBL3813873 | Small molecule | Approval | TTD | 7 | 4 | 4 | Drugs and targets |
| EMACTUZUMAB | CHEMBL3545370 | Antibody | Phase 3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| VIMSELTINIB | CHEMBL5095202 | Small molecule | Phase 3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| PIMICOTINIB | CHEMBL5314535 | Small molecule | Phase 3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| NILOTINIB | CHEMBL255863 | Small molecule | Phase 2 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| MCS-110 | CHEMBL2109512 | Phase 2 | ClinicalTrials.gov and TTD | 2 | 1 | 1 | Drugs and targets | |
| CABIRALIZUMAB | CHEMBL4297713 | Antibody | Phase 1/2 | ClinicalTrials.gov | 3 | 1 | 1 | Drugs and targets |
| AVELUMAB | CHEMBL3833373 | Antibody | Phase 1/2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| IVUXOLIMAB | CHEMBL4297883 | Antibody | Phase 1/2 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| PD-360324 | CHEMBL2109513 | Phase 1/2 | ClinicalTrials.gov | 1 | 1 | 0 | Drugs and targets |
1 to 10 of 13
See all 13 drugsTargets24
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 |
|---|---|---|---|---|---|---|
| CSF1R | Macrophage colony-stimulating factor 1 receptor | 3 | Macrophage colony stimulating factor receptor antagonist; Macrophage colony stimulating factor receptor inhibitor | Approval | https://conductscience.org/library/genes/1436 | Drugs and targets |
| CSF1 | Macrophage colony-stimulating factor 1 | 2 | Macrophage colony-stimulating factor 1 inhibitor | Phase 2 | https://conductscience.org/library/genes/1435 | Drugs and targets |
| ABL1 | Bcr/Abl fusion protein | 1 | Bcr/Abl fusion protein inhibitor; Tyrosine-protein kinase ABL inhibitor | Phase 2 | https://conductscience.org/library/genes/25 | Drugs and targets |
| BCR | Bcr/Abl fusion protein | 1 | Bcr/Abl fusion protein inhibitor | Phase 2 | https://conductscience.org/library/genes/613 | Drugs and targets |
| CD274 | Programmed cell death 1 ligand 1 | 1 | Programmed cell death 1 ligand 1 other | Phase 1/2 | https://conductscience.org/library/genes/29126 | Drugs and targets |
| FLT3 | Receptor-type tyrosine-protein kinase FLT3 | 1 | Tyrosine-protein kinase receptor FLT3 inhibitor | Approval | https://conductscience.org/library/genes/2322 | Drugs and targets |
| KIT | Mast/stem cell growth factor receptor Kit | 1 | Stem cell growth factor receptor inhibitor | Approval | https://conductscience.org/library/genes/3815 | Drugs and targets |
| TNFRSF4 | Tumor necrosis factor receptor superfamily member 4 | 1 | Tumor necrosis factor receptor superfamily member 4 agonist | Phase 1/2 | https://conductscience.org/library/genes/7293 | Drugs and targets |
| TNFRSF9 | Tumor necrosis factor receptor superfamily member 9 | 1 | Tumor necrosis factor receptor superfamily member 9 agonist | Phase 1/2 | https://conductscience.org/library/genes/3604 | Drugs and targets |
| TUBA1A | Tubulin | 1 | Tubulin inhibitor | Phase 1 | https://conductscience.org/library/genes/7846 | Drugs and targets |
1 to 10 of 24
See all 24 targetsTrials14
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 |
|---|---|---|---|---|---|---|---|---|
| NCT02554812 | A Study Of Avelumab In Combination With Other Cancer Immunotherapies In Advanced Malignancies (JAVELIN Medley) | Phase 1/2 | 409 | 2024-07-30 | Advanced Cancer | AVELUMAB, IVUXOLIMAB, UTOMILUMAB | Its record names the disease | ClinicalTrials.gov |
| NCT05059262 | Study of Vimseltinib for Tenosynovial Giant Cell Tumor | Phase 3 | 123 | 2025-02-24 | Tenosynovial Giant Cell Tumor, Pigmented Villonodular Synovitis, Giant Cell Tumor of Tendon Sheath, Tenosynovial Giant Cell Tumor, Diffuse, Tenosynovial Giant Cell Tumor, Localized | VIMSELTINIB | Its record names the disease | ClinicalTrials.gov |
| NCT02371369 | Phase 3 Study of Pexidartinib for Pigmented Villonodular Synovitis (PVNS) or Giant Cell Tumor of the Tendon Sheath (GCT-TS) | Phase 3 | 120 | 2020-01-10 | Pigmented Villonodular Synovitis, Giant Cell Tumors of the Tendon Sheath, Tenosynovial Giant Cell Tumor | PEXIDARTINIB | Its record names the disease | ClinicalTrials.gov |
| NCT02471716 | Study of Cabiralizumab in Patients With Pigmented Villonodular Synovitis / Diffuse Type Tenosynovial Giant Cell Tumor | Phase 1/2 | 66 | 2021-08-31 | Pigmented Villonodular Synovitis, Tenosynovial Giant Cell Tumor | CABIRALIZUMAB | Its record names the disease | ClinicalTrials.gov |
| NCT04488822 | A Study of the Efficacy and Safety of Pexidartinib in Adult Subjects With TGCT | Phase 3 | 40 | 2023-04-27 | Tenosynovial Giant Cell Tumor | PEXIDARTINIB | Open Targets' reading of its record | ClinicalTrials.gov |
| NCT01643850 | MCS110 in Patients With Pigmented Villonodular Synovitis (PVNS) | Phase 2 | 36 | 2020-02-27 | Pigmented Villonodular Synovitis, PVNS, Giant Cell Tumor of the Tendon Sheath, GCCTS, Tenosynovial Giant Cell Tumor Localized or Diffused Type, GCTS | MCS-110 | Its record names the disease | ClinicalTrials.gov |
| NCT04526704 | Study to Evaluate Discontinuation and Re-Treatment in Participants With Tenosynovial Giant Cell Tumor (TGCT) Previously Treated With Pexidartinib | Phase 4 | 32 | 2024-12-30 | Tenosynovial Giant Cell Tumor | PEXIDARTINIB | Open Targets' reading of its record | ClinicalTrials.gov |
| NCT01207492 | Nilotinib in Patients With Relapsed or Metastatic Pigmented Villonodular Synovitis/Tenosynovial Giant Cell Tumor/Diffuse-Type Giant Cell Tumor | Phase 2 | 17 | 2015-06-25 | Pigmented Villonodular Synovitis, Diffuse-type Giant Cell Tumor, Tenosynovial Giant Cell Tumor | NILOTINIB | Its record names the disease | ClinicalTrials.gov |
| NCT04703322 | A Study of Pexidartinib in Tenosynovial Giant Cell Tumor in Japan | Phase 2 | 9 | 2025-03-03 | Tenosynovial Giant Cell Tumor | PEXIDARTINIB | Open Targets' reading of its record | ClinicalTrials.gov |
| NCT01494688 | A Study of RO5509554 as Monotherapy and in Combination With Paclitaxel in Participants With Advanced Solid Tumors | Phase 1 | 217 | Advanced Solid Tumors | EMACTUZUMAB, PACLITAXEL | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 14
See all 14 trialsComparisons in its trials3
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 | Source |
|---|---|---|---|---|---|
| Total - Vimseltinib vs Placebo: deaths from any cause | 1 of 118 | 0 of 39 | 157 | NCT05059262 | Trial comparisons |
| Total - Vimseltinib vs Placebo: serious adverse events | 17 of 118 | 1 of 39 | 157 | NCT05059262 | Trial comparisons |
| Phase 2 FPA008 Dose Expansion Cohort 2A vs Phase 2 FPA008 Dose Expansion Cohort 2B: The Incidence of Investigator-assessed, Confirmed Objective Responses (ORR) Per RECIST 1.1 (Phase 2) | 8 of 32 | 8 of 24 | 56 | NCT02471716 | Trial comparisons |
Papers from its trials19
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 |
|---|---|---|---|
| 42872549 | DERIVED | 1 | PubMed |
| 42473802 | DERIVED | 1 | PubMed |
| 42132251 | DERIVED | 1 | PubMed |
| 41796601 | DERIVED | 1 | PubMed |
| 40841499 | DERIVED | 1 | PubMed |
| 39287124 | DERIVED | 1 | PubMed |
| 38843860 | DERIVED | 1 | PubMed |
| 37593881 | DERIVED | 1 | PubMed |
| 36001000 | DERIVED | 1 | PubMed |
| 35932628 | DERIVED | 1 | PubMed |
1 to 10 of 19
See all 19 papers from its trialsData dates
The date of each source this release read.
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/EFO_1001106
GET https://conductscience.org/api/diseases/v1/EFO_1001106/targets?page=2