Diffuse tenosynovial giant cell tumor (D-TGCT) is a specific presentation of TGCT with multinodular proliferation in larger joints and a tendency toward local tissue destruction. A structured, evidence-based approach guides therapy in this setting.
D-TGCT forms multiple nodules that proliferate in the larger joints — most commonly the knees, accounting for approximately 75% of cases — and is associated with a significant risk of local tissue destruction. This diffuse pattern defines a population requiring a distinct therapeutic strategy.
Oral systemic therapy is central to this protocol — including pexidartinib, which holds dedicated FDA approval for symptomatic TGCT, and imatinib as an additional oral option.
DOI: 10.56875/2589-0646.1032
D-TGCT forms multiple nodules that proliferate in the larger joints, such as the knees (75%), and are likely to be locally destructive.
It was approved by FDA in 2019 for the treatment of adults with symptomatic TGCT who show severe morbidity or functional limitations that cannot be improved with surgery, based on the results of ENLIVEN trial.
Imatinib, a multi-kinase inhibitor, was the first CSFR1 blocker showing activity against TGCT, with a complete response (CR) reported in a case.
It demonstrated an ORR of 39% at week 25 compared to placebo.
Moreover, it improved the functional outcomes, including the mean range of motion and physical function and decreased stiffness.
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