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Home/Rare Diseases/Tenosynovial Giant Cell Tumor

Connective Tissue & Musculoskeletal

Tenosynovial Giant Cell Tumor (PVNS) Clinical Trials

Also called Pigmented Villonodular Synovitis, PVNS, Giant Cell Tumor of the Tendon Sheath, Diffuse Tenosynovial Giant Cell Tumor, Localized Tenosynovial Giant Cell Tumor, Nodular Tenosynovitis

Tenosynovial giant cell tumor grows from the thin lining of a joint, a tendon sheath or a bursa. In a small share of the tumor's cells, a swap between chromosomes 1 and 2 involving the CSF1 gene makes those cells overproduce a signal called CSF1. That signal draws in macrophages and other inflammatory cells, which make up most of the tumor (NORD; West et al., 2006).

In plain words, tenosynovial giant cell tumor is a rare growth in the lining of a joint or tendon, most often in the knee or a finger. It is usually not cancer and does not spread, but it can cause pain, swelling and stiffness and can damage the joint over time.

Tenosynovial Giant Cell Tumor

Where treatments stand, from ClinicalTrials.gov and FDA labels

Also 2 studies with no phase, like observational studies, and 1 phase 4 study following medicines after approval.

FDA review0

No decision pending. FDA calendar

FDA-approved medicines2

Romvimza · Turalio

Tenosynovial Giant Cell Tumor has 2 FDA-approved medicines, 7 open or active clinical trials (5 recruiting), and no pending FDA decision.

Labels checked October 21, 2026. Corticosteroids and immune globulins count once each.Download the data

More about Tenosynovial Giant Cell Tumor

TGCT comes in 2 forms that look the same under the microscope. The localized form is a single, well-defined nodule, most often in a finger or toe, and surgery often cures it. The diffuse form, once called pigmented villonodular synovitis (PVNS), spreads through the lining of a large joint, usually the knee, and it comes back more often. NORD reports recurrence after surgery of about 10-15% for the localized form and 50-70% for the diffuse form.

Keep reading about Tenosynovial Giant Cell Tumor

MRI is the main test, and a biopsy is sometimes needed. Surgery to remove the affected lining is the standard first treatment, and people without symptoms may simply be watched with MRI. For people whose surgery could worsen function or cause severe harm, the FDA has approved 2 medicines that block the CSF1 receptor: Turalio (pexidartinib) in 2019 and Romvimza (vimseltinib) in 2025.

What are the symptoms of Tenosynovial Giant Cell Tumor?

These are common symptoms of Tenosynovial Giant Cell Tumor, and how strong they are varies from person to person.

  • Pain in one joint, most often the knee, ankle or hip
  • Swelling of the joint, sometimes painless at first
  • Stiffness and less range of motion
  • Warmth or tenderness over the joint
  • Locking, catching or popping, or a joint that feels unstable
  • A slow-growing lump on a finger, toe or tendon in the localized form

Who gets Tenosynovial Giant Cell Tumor?

TGCT is usually diagnosed between ages 25 and 50, with a median age of about 40 according to NORD, though children and older adults can have it. The localized form is slightly more common in women, while the diffuse form affects women and men about equally (NORD). Diffuse TGCT most often involves the knee, then the ankle and hip, and the localized form most often a finger or toe. Diagnosis is often delayed by 3-4 years (NORD).

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Which medicines are FDA-approved for Tenosynovial Giant Cell Tumor?

There are currently 2 FDA-approved medications for Tenosynovial Giant Cell Tumor.

vimseltinib
Deciphera Pharmaceuticals (part of Ono Pharmaceutical)
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pexidartinib
Daiichi Sankyo
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Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.

How to get help paying for Tenosynovial Giant Cell Tumor treatment

Charity funds and drugmaker programs for Tenosynovial Giant Cell Tumor, checked at the source.

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Side effects people have reported to the FDA

Side effects people have reported to the FDA

Real-world side effect reports from the FDA Adverse Event Reporting System (FAERS). Includes both FDA-approved drugs and investigational therapies from active clinical trials. Click any drug to see what patients reported.

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Diagnosis and genetic testing

How Tenosynovial Giant Cell Tumor is diagnosed

Not inherited. The CSF1 change is found only in some of the tumor's cells, and NORD says no genetic, environmental or lifestyle risk factor has been shown.

Trusted Tenosynovial Giant Cell Tumor resources

Reputable organizations and medical references for learning more about Tenosynovial Giant Cell Tumor, including disease registries, foundation resources, and clinical guidelines.

Active clinical trials for Tenosynovial Giant Cell Tumor

Use this Tenosynovial Giant Cell Tumor clinical trial finder to see the 5 studies recruiting patients and 2 opening soon in the United States and worldwide, with eligibility criteria in plain English. Of these, 5 test a treatment and 2 are observational studies or registries; each card says which. Every trial comes from ClinicalTrials.gov.

Jump to the trial list, the site map or the pipeline timeline.

Note: Trial recruitment statuses on ClinicalTrials.gov may not immediately reflect recent FDA decisions, sponsor announcements, or enrollment changes. Always confirm a trial's current status directly with the study coordinator before making plans.

7 active trials worldwide
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RecruitingTreatment trialPhase 2Recently updatedNCT07499362

Study of Pimicotinib in Japanese Participants With Tenosynovial Giant Cell Tumor (TGCT) (J-MANEUVER)

Intervention: Pimicotinib

Sponsor: Merck Healthcare KGaA, Darmstadt, Germany, an affiliate of Merck KGaA, Darmstadt, Germany · Merck KGaA, Darmstadt, Germany

The primary purpose of this study is to assess the tolerability, pharmacokinetics, and efficacy of pimicotinib in Japanese participants with TGCT

Ages 18 Years+4 locations
Started Mar 2026Updated 6 days agoEst. Aug 2028 (~1y 9m)
Not yet recruitingTreatment trialPhase 2Recently updatedNCT07832747

A Study of DCC-3014 to Evaluate the Efficacy, Safety, and Pharmacokinetics in Subjects With TGCT

Intervention: DCC-3014

Sponsor: Ono Pharmaceutical Co., Ltd.

To evaluate the efficacy, safety, and pharmacokinetics of DCC-3014 in subjects with Tenosynovial Giant Cell Tumor

Ages 18 Years+1 location
Expected to start Oct 2026Updated 1 month agoEst. Aug 2028 (~1y 10m)
RecruitingObservational study, no treatment givenUpdated a few months agoNCT07075471

MOVE: Tenosynovial Giant Cell Tumor (TGCT) Real-World Participant Experience With Vimseltinib

Sponsor: Deciphera Pharmaceuticals, LLC

The goal of this observational study is to better understand real-world use of vimseltinib, treatment patterns, and health-related quality of life in participants treated with vimseltinib for TGCT.

Ages 18 Years+1 location
Started Sep 2025Updated 3 months agoEst. Sep 2027 (~11 months)
Not yet recruitingTreatment trialPhase 2Updated a few months agoNCT07707882

Neoadjuvant Pimicotinib Combined With Surgery Versus Upfront Surgery for Diffuse Tenosynovial Giant Cell Tumor

Intervention: Pimicotinib, Tumor resection surgery

Sponsor: Second Affiliated Hospital, Zhejiang University, School of Medicine

Tenosynovial giant cell tumor (TGCT) is a rare neoplasm predominantly occurring in young and middle-aged adults, characterized by high recurrence and high disability rates. Surgery represents the first-line treatment at present. Although surgical resection can eradicate lesions, ...

Ages 18 Years+1 location
Expected to start Oct 2027Updated 3 months agoEst. Sep 2029 (~2y 11m)
RecruitingTreatment trialPhase 1No updates in a whileNCT04192344

A Study to Assess the Safety, Tolerability, and Pharmacokinetics of ABSK-021 in Patients With Advanced Solid Tumor

Intervention: ABSK021

Sponsor: Abbisko Therapeutics Co, Ltd

This is an open-label phase 1 study to determine the safety and tolebility of oral ABSK021 in patients with advanced solid tumor as well as the Recommended Phase 2 dose (RP2D) of oral ABSK021. Preliminary antitumor activity will also be assessed.

Ages 18 Years+17 locations
Started Jan 2020Updated 7 months agoEst. Dec 2026 (~2 months)
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Active trial locations10 cities in the US
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Trial Pipeline

Jan 2021 to Oct 2031
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Data from ClinicalTrials.gov, U.S. National Library of Medicine.
Always talk to your doctor before considering a clinical trial.

Companies developing Tenosynovial Giant Cell Tumor treatments

Drugmakers that list Tenosynovial Giant Cell Tumor in their rare disease portfolio, 2 in all.

Questions people ask about Tenosynovial Giant Cell Tumor