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tenosynovial giant cell tumor, diffuse type (TGCT) - Clinical Profile, Genes & Research | Kisho | Kisho

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for tenosynovial giant cell tumor, diffuse type. An additional 4 compounds hold orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for tenosynovial giant cell tumor, diffuse type, 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 tenosynovial giant cell tumor, diffuse type. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor	Designated	Exclusivity End	Designation Status
Cabiralizumab	Cabiralizumab	Five Prime Therapeutics, Inc.	2016	—	Withdrawn
Lacnotuzumab	Lacnotuzumab	Novartis Pharmaceuticals Corp	2014	—	Withdrawn
recombinant humanized monoclonal antibody of the immunoglobulin G1 subclass directed against colony stimulating factor-1 receptor expressed on macrophages	recombinant humanized monoclonal antibody of the immunoglobulin G1 subclass directed against colony stimulating factor-1 receptor expressed on macrophages	Genentech, Inc.	2014	—	Withdrawn
TURALIO	pexidartinib	Daiichi Sankyo, Inc.	2014	2026	Designated (drug approved for other indication)

TURALIO is referenced in active clinical trials for tenosynovial giant cell tumor, diffuse type (designated 2014).

Gene therapy approaches for tenosynovial giant cell tumor, diffuse type have been reported in the published literature.

Clinical Trials

5 trials found

Explore Trials

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.

5 clinical trials registered. Interventions under study include drug therapy and procedural interventions. Pipeline includes 2 PHASE3, 2 PHASE2, 1 PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT04703322](https://clinicaltrials.gov/study/NCT04703322)	A Study of Pexidartinib in Tenosynovial Giant Cell Tumor in Japan	PHASE2	Daiichi Sankyo Co., Ltd.	UNKNOWN
[NCT05804045](https://clinicaltrials.gov/study/NCT05804045)	Study of Pimicotinib (ABSK021) for Tenosynovial Giant Cell Tumor (MANEUVER)	PHASE3	Abbisko Therapeutics Co, Ltd	UNKNOWN
[NCT03069469](https://clinicaltrials.gov/study/NCT03069469)	Study of Vimseltinib (DCC-3014) in Patients With Advanced Tumors and Tenosynovial Giant Cell Tumor	PHASE1	Deciphera Pharmaceuticals, LLC	ACTIVE_NOT_RECRUITING
[NCT05059262](https://clinicaltrials.gov/study/NCT05059262)	Study of Vimseltinib for Tenosynovial Giant Cell Tumor	PHASE3	Deciphera Pharmaceuticals, LLC	ACTIVE_NOT_RECRUITING
[NCT07707882](https://clinicaltrials.gov/study/NCT07707882)	Neoadjuvant Pimicotinib Combined With Surgery Versus Upfront Surgery for Diffuse Tenosynovial Giant Cell Tumor	PHASE2	Second Affiliated Hospital, Zhejiang University, School of Medicine	NOT_YET_RECRUITING

190 publications have been identified in PubMed for tenosynovial giant cell tumor, diffuse type. Research spans Case Report / Case Series (44%), Review / Meta-Analysis (17%), and Clinical Trial Publication (12%).

Research Type	Count	% of Total
Patient case studies	84	44%
Research summaries	32	17%
Clinical study results	22	12%
Laboratory research	17	9%
Disease patterns and progression	14	7%
Testing and diagnosis research	11

Recent Publications

Lin Y (2026). [PMID: 41578522](https://pubmed.ncbi.nlm.nih.gov/41578522/). *Medicine (Baltimore)*. [Case Report / Case Series]
Rachidi S (2026). [PMID: 42170131](https://pubmed.ncbi.nlm.nih.gov/42170131/). *Cureus*. [Case Report / Case Series]
Mosher H (2026). [PMID: 41523664](https://pubmed.ncbi.nlm.nih.gov/41523664/). *JB & JS open access*. [Clinical Trial Publication]
Falke I (2026). [PMID: 41391893](https://pubmed.ncbi.nlm.nih.gov/41391893/). *Semin Radiat Oncol*. [Review / Meta-Analysis]
Wang C (2026). [PMID: 42232324](https://pubmed.ncbi.nlm.nih.gov/42232324/). *Front Surg*. [Case Report / Case Series]
van der Linde RM (2026). [PMID: 41082975](https://pubmed.ncbi.nlm.nih.gov/41082975/). *Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc*. [Clinical Trial Publication]
Conway AP (2026). [PMID: 41557367](https://pubmed.ncbi.nlm.nih.gov/41557367/). *Expert review of anticancer therapy*. [Review / Meta-Analysis]
Konstantinou E (2026). [PMID: 41976400](https://pubmed.ncbi.nlm.nih.gov/41976400/). *Cancers (Basel)*. [Case Report / Case Series]
Oliveira V (2026). [PMID: 41351629](https://pubmed.ncbi.nlm.nih.gov/41351629/). *Pediatr Radiol*. [Diagnostic / Biomarker]
De Smet M (2026). [PMID: 41791925](https://pubmed.ncbi.nlm.nih.gov/41791925/). *Bulletin du cancer*. [Case Report / Case Series]

Kisho

tenosynovial giant cell tumor, diffuse type

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

[Tenosyovial giant cell tumor (TGCT): From the physiopathology to the emergence of new targeted therapies].

Two cases of giant cell tumours of the orbit: one case of giant cell tumor associated with Paget's disease of bone and one case of tenosynovial giant cell tumor.

tenosynovial giant cell tumor, diffuse type

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

[Tenosyovial giant cell tumor (TGCT): From the physiopathology to the emergence of new targeted therapies].

Two cases of giant cell tumours of the orbit: one case of giant cell tumor associated with Paget's disease of bone and one case of tenosynovial giant cell tumor.