Overview
Tenosynovial giant cell tumors (TGCT) are locally aggressive tumors of the synovium, bursae, and tendon sheaths. The diffuse type (D-TGCT, formerly PVNS) causes progressive joint destruction and significant disability. TGCT is driven by overexpression of colony-stimulating factor 1 (CSF1). Pexidartinib (Turalio) was approved in 2019 for symptomatic TGCT not amenable to surgery. SpringWorks Therapeutics is developing pimicotinib (a selective CSF1R inhibitor) as a pipeline therapy.
Also known as:
Key symptoms:
Joint pain, often in the knee, hip, or ankleSwelling or puffiness around a jointStiffness that makes it hard to fully bend or straighten the jointA visible or palpable lump near a tendon, especially in the fingers or handFeeling of warmth around the affected jointLocking or catching sensation in the joint during movementReduced range of motion in the affected jointJoint giving way or feeling unstableGradual worsening of symptoms over months to yearsBone erosion or damage seen on imaging in advanced cases
Clinical phenotype terms (24)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsMerck Healthcare KGaA, Darmstadt, Germany, an affiliate of Merck KGaA, Darmstadt, Germany — PHASE2
Nantes University Hospital — NA
Shanghai Jiao Tong University Affiliated Sixth People's Hospital — PHASE4
Deciphera Pharmaceuticals, LLC
Zixin Deng — NA
SynOx Therapeutics Limited — PHASE3
Abbisko Therapeutics Co, Ltd — PHASE3
Deciphera Pharmaceuticals, LLC — PHASE3
Daiichi Sankyo Co., Ltd. — PHASE2
Daiichi Sankyo
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableTURALIO
TURALIO is indicated for the treatment of adult patients with symptomatic tenosynovial giant cell tumor (TGCT) associated with severe morbidity or functional limitations and not amenable to improvemen…
TURALIO is indicated for the treatment of adult patients with symptomatic tenosynovial giant cell tumor (TGCT) associated with severe morbidity or functional limitations and not amenable to improvement with surgery.
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
1 resourcesTravel Grants
No travel grants are currently matched to Tenosynovial giant cell tumor.
Community
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Start the conversation →Latest news about Tenosynovial giant cell tumor
Disease timeline:
New recruiting trial: Accuracy of Indocyanine Green (ICG) Fluorescence Imaging in Tenosynovial Giant Cell Tumor Surgery
A new clinical trial is recruiting patients for Tenosynovial giant cell tumor
New recruiting trial: A Long-term Study Evaluating Hepatotoxicity Associated With TURALIO™ (Pexidartinib) Treatment
A new clinical trial is recruiting patients for Tenosynovial giant cell tumor
New recruiting trial: A Study to Assess the Safety, Tolerability, and Pharmacokinetics of ABSK-021 in Patients With Advanced Solid Tumor
A new clinical trial is recruiting patients for Tenosynovial giant cell tumor
New recruiting trial: Tenosynovial Giant Cell Tumor (TGCT) Real-World Participant Experience With Vimseltinib
A new clinical trial is recruiting patients for Tenosynovial giant cell tumor
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Do I have the localized or diffuse form of TGCT, and how does that affect my treatment options?,Is surgery the best first step for me, and what type of surgery would you recommend?,What is the chance that my tumor will come back after treatment, and how will we monitor for that?,Am I a candidate for pexidartinib (Turalio), and what are the risks and benefits for my specific situation?,Should I be seen at a specialized center with experience in TGCT, and can you refer me to one?,How much joint damage has already occurred, and could I eventually need a joint replacement?,Are there any clinical trials I should consider for my type of TGCT?
Common questions about Tenosynovial giant cell tumor
What is Tenosynovial giant cell tumor?
Tenosynovial giant cell tumors (TGCT) are locally aggressive tumors of the synovium, bursae, and tendon sheaths. The diffuse type (D-TGCT, formerly PVNS) causes progressive joint destruction and significant disability. TGCT is driven by overexpression of colony-stimulating factor 1 (CSF1). Pexidartinib (Turalio) was approved in 2019 for symptomatic TGCT not amenable to surgery. SpringWorks Therapeutics is developing pimicotinib (a selective CSF1R inhibitor) as a pipeline therapy.
How is Tenosynovial giant cell tumor inherited?
Tenosynovial giant cell tumor follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Tenosynovial giant cell tumor typically begin?
Typical onset of Tenosynovial giant cell tumor is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Tenosynovial giant cell tumor?
Yes — 10 recruiting clinical trials are currently listed for Tenosynovial giant cell tumor on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Tenosynovial giant cell tumor?
13 specialists and care centers treating Tenosynovial giant cell tumor are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Tenosynovial giant cell tumor?
1 patient support program are currently tracked on UniteRare for Tenosynovial giant cell tumor. See the treatments and support programs sections for copay assistance, eligibility, and contact details.