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Tenosynovial giant cell tumor (TGCT)
Tenosynovial giant cell tumor (TGCT), previously known as PVNS, are relatively rare neoplasms that arise from the synovial lining of joints, bursae, and tendon sheaths
Prevalence
2-5/100,000
6,620-16,550
US Estimated
9,000-22,500
Europe Estimated
Age of Onset
ICD-10
D48.1
Rare View
The most common form of rare synovial tumors, TGCT is caused by overexpression of the CSF1 gene, leading to abnormal macrophage-like cell accumulation within joint tissue. Patients typically present with chronic joint pain, recurrent effusions, and limited range of motion — most often affecting the knee in the diffuse form, or fingers and wrist in the localized form. The presence of hemosiderin deposits on MRI and high recurrence rates in diffuse-type TGCT distinguish it from other benign joint lesions.
5 Facts you should know
FACT
TGCT, previously known as pigmented villonodular synovitis (PVNS) or giant cell tumor of the tendon sheath (GCT-TS), is a locally aggressive neoplasm associated with overexpression of the CSF1 gene. It presents in two forms: localized/nodular TGCT and diffuse TGCT, the latter carrying a substantially higher risk of recurrence
FACT
TGCT typically presents in young to middle adulthood, with a median age of approximately 33 years, and an estimated annual incidence of 11 to 50 cases per million people. Recurrence following surgical resection is particularly common in diffuse-type TGCT, where rates approaching 70% have been reported
FACT
In an international, prospective patient registry, over 90% of individuals with TGCT reported pain and 85% reported limited range of motion, underscoring the substantial impact of this disease on physical function and quality of life
FACT
When inadequately managed, diffuse TGCT can cause progressive cartilage destruction, bone erosion, and joint degeneration, with severe cases ultimately requiring joint replacement or amputation
FACT
Surgical resection is the standard of care for TGCT but is not always curative, and repeated resections can lead to increased morbidity. For patients in whom surgery may result in severe morbidity or worsening functional limitation, CSF1R-targeted tyrosine kinase inhibitors represent an FDA-approved systemic treatment option
Interest over time
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Common signs & symptoms
Joint pain — often the earliest and most consistent complaint
Joint swelling and effusion — may be significant in diffuse
TGCT Limited range of motion — stiffness and restricted movement
Locking or catching sensation — particularly with knee involvement
Palpable soft tissue mass — more common in localized form (fingers, wrist)
Joint tenderness on palpation
Joint warmth
Bone erosion — seen in advanced diffuse TGCT on imaging
Current treatments
Surgical Synovectomy
The primary treatment for both forms. Arthroscopic or open synovectomy aims for complete removal of affected synovium. Complete resection is often achievable in localized TGCT; diffuse TGCT recurs in up to 50% of cases after surgery alone due to the extent of synovial involvement.
Vimseltinib (Romvimza)
FDA-approved February 2025 for adult patients with symptomatic TGCT not amenable to improvement with surgery. Vimseltinib is a selective CSF1R inhibitor taken orally twice weekly. Approval was based on the Phase 3 MOTION trial, which demonstrated a 40% overall response rate versus 0% in the placebo arm, with meaningful improvements in tumor volume, pain, and physical function. Notably, vimseltinib showed no treatment-related serious hepatotoxicity in clinical trials — a significant differentiator from earlier systemic options — making it a preferred choice for eligible patients requiring systemic therapy.
Pexidartinib (Turalio)
FDA-approved 2019 as the first systemic therapy for symptomatic TGCT not amenable to surgery. A CSF1R inhibitor with demonstrated efficacy; carries a boxed warning for serious and potentially fatal hepatotoxicity and requires enrollment in a REMS program.
Active surveillance
Appropriate for small, asymptomatic localized TGCT with no functional impairment. Regular monitoring with MRI is recommended.
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| A Study of Pexidartinib in Tenosynovial Giant Cell Tumor in Japan | This phase 2, multicenter, two-part, open-label, single-arm study will be conducted in Japan and will evaluate the safety, tolerability, pharmacokinetics (PK), and efficacy of pexidartinib in adult participants with symptomatic tenosynovial giant cell tumor (TGCT) associated with severe morbidity or... | Phase 2 | Active | Drug: Pexidartinib | More Info |
| Tenosynovial Giant Cell Tumor (TGCT) Real-World Participant Experience With Vimseltinib | 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. This study will collect data in an observational disease registry involving no intervention to the pa... | Recruiting | More Info | ||
| Study of Pimicotinib (ABSK021) for Tenosynovial Giant Cell Tumor (MANEUVER) | The goal of this clinical trial is to assess the efficacy and safety of Pimicotinib (ABSK021) in patients with Tenosynovial Giant Cell Tumor (TGCT). The main questions it aims to answer are: * Whether the Pimicotinib(ABSK021) works well in patients with TGCT. * Whether the Pimicotinib(ABSK021) is s... | Phase 3 | Active | Drug: Pimicotinib(ABSK021) Drug: Placebo | More Info |
| Study of Vimseltinib (DCC-3014) in Patients With Advanced Tumors and Tenosynovial Giant Cell Tumor | This is a multicenter, open-label Phase 1/2 study of vimseltinib in patients with malignant solid tumors and tenosynovial giant cell tumor (TGCT). There will be 2 distinct parts in this study: Dose Escalation (Phase 1) and Expansion (Phase 2). Phase 1 will enroll both malignant solid tumor and TGCT... | Phase 1, Phase 2 | Active | Drug: Vimseltinib | More Info |
| Phase I Trial of TURALIO(R) (Pexidartinib, PLX3397) in Children and Young Adults With Refractory Leukemias and Refractory Solid Tumors Including Neurofibromatosis Type 1 (NF1) Associated Plexiform Neurofibromas (PN) and Tenosynovial Giant Cell Tumor ... | Background: \- Some people with cancer have solid tumors. Others have refractory leukemia. This may not go away after treatment. Researchers want to see if a drug called TURALIO(R) can shrink tumors or stop them from growing. Objectives: \- To find the highest safe dose and side effects of TURALI... | Phase 1 | Recruiting | Drug: TURALIO(R) | More Info |
| A Study of the Efficacy and Safety of Pexidartinib in Adult Subjects With TGCT | This study will assess pexidartinib in adult participants with symptomatic TGCT that is associated with severe morbidity or functional limitations and not amendable to improvement with surgery. | Phase 3 | Active | Drug: Pexidartinib | More Info |
| Accuracy of Indocyanine Green (ICG) Fluorescence Imaging in Tenosynovial Giant Cell Tumor Surgery | This study evaluates the diagnostic accuracy of Indocyanine Green (ICG) fluorescence imaging in visualizing Tenosynovial Giant Cell Tumor (TGCT) lesions during surgery. Patients diagnosed with TGCT will receive an intravenous injection of ICG prior to the operation to label tumor tissues. During the... | Phase 4 | Recruiting | Drug: Indocyanine Green | More Info |
| Study of Vimseltinib for Tenosynovial Giant Cell Tumor | This is a multicenter Phase 3 clinical study, which aims to evaluate the effectiveness of an investigational drug called vimseltinib for the treatment of tenosynovial giant cell tumor (TGCT) in cases where surgical removal of the tumor is not an option. The study consists of two parts. In Part 1, e... | Phase 3 | Active | Drug: Vimseltinib Drug: Placebo | More Info |
| Study of Emactuzumab for Tenosynovial Giant Cell Tumor (TGCT) | This is a multicenter, Phase 3, randomised, double-blind, placebo-controlled study, which aims to evaluate the efficacy and safety of the investigational drug emactuzumab for the treatment of patients with localized or diffuse TGCT where surgical removal of the tumor is not viewed as an option. The... | Phase 3 | Active | Drug: Emactuzumab Drug: Placebo | More Info |