Rare Oncology News
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5 Facts you should know about
Tenosynovial giant cell tumor (TGCT)
1
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
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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
3
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
4
When inadequately managed, diffuse TGCT can cause progressive cartilage destruction, bone erosion, and joint degeneration, with severe cases ultimately requiring joint replacement or amputation
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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