Medical Management of Tenosynovial Giant Cell Tumor

Diffuse tenosynovial giant cell tumor (D-TGCT) is a benign neoplasm with locally aggressive potential of the synovium, bursae, and tendon sheaths. This review summarizes the current treatment landscape for D-TGCT, with a focus on systemic therapies.

Surgery is the primary treatment option for tenosynovial giant cell tumor (TGCT), but there is a high risk of recurrence and associated morbidity, particularly for patients with advanced D-TGCT. Systemic therapies targeting the colony-stimulating factor 1 receptor (CSF1R) have resulted in positive tumor response, improved function, and decreased symptoms. For an alternative to surgery, the CSF1R inhibitors pexidartinib and vimseltinib are approved in the United States for TGCT, and other CSF1R inhibitors are in clinical development. CSF1R inhibitors represent a significant evolution in therapeutic strategies for D-TGCT. The potential risks and benefits of available treatments should be carefully considered in collaboration with a bone tumor-experienced, multidisciplinary team to determine the best course of care. Increased D-TGCT awareness and support through patient advocacy groups have helped to reshape the patient journey.