Tenosynovial giant cell tumor
diseaseOn this page
Also known as fibrous histiocytoma of tendon sheathgiant cell neoplasm of tendon sheathgiant cell neoplasm of Tenosynoviumgiant cell neoplasm of the Tenosynoviumgiant cell tumor of tendon sheathgiant cell tumor of Tenosynoviumgiant cell tumor of the Tenosynoviumgiant cell tumour of the Tenosynoviumtendon sheath giant cell neoplasmtendon sheath giant cell tumortendon sheath giant cell tumourtenosynovial giant cell neoplasm
Summary
Tenosynovial giant cell tumor (MONDO:0002522) is a cancer and 19 clinical trials. Molecularly, CSF1 Overexpression confers sensitivity to Pexidartinib in Tenosynovial Giant Cell Tumor (CIViC Level B). Top therapeutic interventions include pexidartinib, indocyanine green acid form, and nilotinib. A subtype of giant cell tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 19
- Precision-medicine evidence (CIViC): 1 subtype–drug association
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | tenosynovial giant cell tumor |
| Mondo ID | MONDO:0002522 |
| EFO | EFO:1000562 |
| DOID | DOID:314 |
| NCIT | C3402 |
| SNOMED CT | 310605004 |
| UMLS | C1318543 |
| MedGen | 232927 |
| Anatomy (UBERON) | UBERON:0000304 |
| Is cancer (heuristic) | yes |
Also known as: fibrous histiocytoma of tendon sheath · giant cell neoplasm of tendon sheath · giant cell neoplasm of Tenosynovium · giant cell neoplasm of the Tenosynovium · giant cell tumor of tendon sheath · giant cell tumor of Tenosynovium · giant cell tumor of the Tenosynovium · giant cell tumour of the Tenosynovium · tendon sheath giant cell neoplasm · tendon sheath giant cell tumor · tendon sheath giant cell tumour · tenosynovial giant cell neoplasm · tenosynovial giant cell tumor
Disease family
This is a subtype of giant cell tumor. 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 etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › giant cell tumor › tenosynovial giant cell tumor
Related subtypes (1): malignant giant cell tumor
Subtypes (3): tenosynovial giant cell tumor, localized type, malignant tenosynovial giant cell tumor, tenosynovial giant cell tumor, diffuse type
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
Drugs indicated for this disease
0 approved, 2 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Pimicotinib | Phase 3 (in late-stage trials) |
| Vimseltinib | Phase 3 (in late-stage trials) |
Clinical trials & evidence
Clinical trials
Clinical trials: 19.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 6 |
| PHASE3 | 4 |
| PHASE1 | 3 |
| PHASE4 | 2 |
| PHASE1/PHASE2 | 2 |
| Not specified | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT07315841 | PHASE4 | RECRUITING | Accuracy of Indocyanine Green (ICG) Fluorescence Imaging in Tenosynovial Giant Cell Tumor Surgery |
| NCT04526704 | PHASE4 | COMPLETED | Study to Evaluate Discontinuation and Re-Treatment in Participants With Tenosynovial Giant Cell Tumor (TGCT) Previously Treated With Pexidartinib |
| NCT04488822 | PHASE3 | ACTIVE_NOT_RECRUITING | A Study of the Efficacy and Safety of Pexidartinib in Adult Subjects With TGCT |
| NCT05059262 | PHASE3 | ACTIVE_NOT_RECRUITING | Study of Vimseltinib for Tenosynovial Giant Cell Tumor |
| NCT05804045 | PHASE3 | ACTIVE_NOT_RECRUITING | Study of Pimicotinib (ABSK021) for Tenosynovial Giant Cell Tumor (MANEUVER) |
| NCT02371369 | PHASE3 | COMPLETED | Phase 3 Study of Pexidartinib for Pigmented Villonodular Synovitis (PVNS) or Giant Cell Tumor of the Tendon Sheath (GCT-TS) |
| NCT03069469 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Study of Vimseltinib (DCC-3014) in Patients With Advanced Tumors and Tenosynovial Giant Cell Tumor |
| NCT04703322 | PHASE2 | ACTIVE_NOT_RECRUITING | A Study of Pexidartinib in Tenosynovial Giant Cell Tumor in Japan |
| NCT07499362 | PHASE2 | RECRUITING | Study of Pimicotinib in Japanese Participants With Tenosynovial Giant Cell Tumor (TGCT) (J-MANEUVER) |
| NCT01207492 | PHASE2 | COMPLETED | Nilotinib in Patients With Relapsed or Metastatic Pigmented Villonodular Synovitis/Tenosynovial Giant Cell Tumor/Diffuse-Type Giant Cell Tumor |
| NCT02471716 | PHASE1/PHASE2 | COMPLETED | Study of Cabiralizumab in Patients With Pigmented Villonodular Synovitis / Diffuse Type Tenosynovial Giant Cell Tumor |
| NCT04731675 | PHASE2 | COMPLETED | An Open-Label Study of Intra-articular AMB-05X Injections in Subjects With Tenosynovial Giant Cell Tumor of the Knee |
| NCT04938180 | PHASE2 | TERMINATED | A Phase 2 Study of Intravenous AMB-05X in Tenosynovial Giant Cell Tumor Patients |
| NCT05349643 | PHASE2 | COMPLETED | A Study to Evaluate Safety and Efficacy of AMB-05X Injections in Subjects With TGCT |
| NCT04192344 | PHASE1 | RECRUITING | A Study to Assess the Safety, Tolerability, and Pharmacokinetics of ABSK-021 in Patients With Advanced Solid Tumor |
| NCT01804530 | PHASE1 | TERMINATED | Phase 1 Study of PLX7486 as Single Agent in Patients With Advanced Solid Tumors |
| NCT02673736 | PHASE1 | TERMINATED | A Study of PLX73086 in Advanced Solid Tumors and Locally Advanced or Refractory Tenosynovial Giant Cell Tumor |
| NCT04635111 | Not specified | RECRUITING | A Long-term Study Evaluating Hepatotoxicity Associated With TURALIO™ (Pexidartinib) Treatment |
| NCT07075471 | Not specified | RECRUITING | Tenosynovial Giant Cell Tumor (TGCT) Real-World Participant Experience With Vimseltinib |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| PEXIDARTINIB | 4 | 4 |
| INDOCYANINE GREEN ACID FORM | 4 | 2 |
| NILOTINIB | 4 | 1 |
| PIMICOTINIB | 3 | 2 |
| VIMSELTINIB | 3 | 2 |
| CABIRALIZUMAB | 2 | 1 |
Precision-medicine subtype map (CIViC)
Drug × molecular subtype: 1 predictive associations from 1 curated evidence items.
| Molecular subtype | Therapy | Effect | Level | CIViC |
|---|---|---|---|---|
| CSF1 Overexpression | Pexidartinib | Sensitivity/Response | CIViC B | EID1969 |