Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive disease that can cause chronic pain, joint dysfunction, reduced mobility, and significant impacts on quality of life, particularly in patients with diffuse disease. In this expert discussion, Dr William Tap and Dr Emanuela Palmerini explore the evolving management of TGCT, including the biologic role of the CSF1/CSF1R pathway, challenges in diagnosis and disease monitoring, and the importance of multidisciplinary, patient-centered care. They also discuss the growing role of CSF1R-targeted therapies, treatment selection, toxicity management, drug holidays, and practical approaches to optimizing long-term outcomes for patients living with TGCT.
Topics Covered
Understanding localized vs diffuse TGCT
Biology of the CSF1/CSF1R pathway
Diagnostic approaches and the role of MRI and biopsy
Multidisciplinary care and shared decision-making
Impact of TGCT on function, mobility, and quality of life
Current and emerging CSF1R-targeted therapies
Pexidartinib and vimseltinib: clinical considerations and toxicity management
Response assessment beyond imaging
Drug holidays, retreatment strategies, and long-term follow-up
Future directions in TGCT research and treatment
Faculty
William Tap, MD
Emanuela Palmerini, MD, PhD
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