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Tenosynovial giant cell tumor (TGCT) is a non-malignant tumor defined histologically as inclusions of “osteoclast-like” multinucleated giant cells, hemosiderin, and macrophages. [1] This histology can present one of 2 clinically distinct ways. TGCT tumors often develop from the lining of joints (also known as synovial tissue).
Vimseltinib is an investigational new drug that is being evaluated to treat tenosynovial giant cell tumor. [1] It is a macrophage colony-stimulating factor receptor antagonist. [ 2 ]
Pexidartinib, sold under the brand name Turalio, is a kinase inhibitor drug for the treatment of adults with symptomatic tenosynovial giant cell tumor (TGCT) associated with severe morbidity or functional limitations and not amenable to improvement with surgery.
In December 2023, Abbisko Therapeutics entered into a licensing agreement for pimicotinib in all indications for China rights with Merck KGaA. [2] [3] [4]In April 2023, a global phase III, randomized, double-blind, placebo-controlled, multicenter clinical trial designed to evaluate the safety and efficacy of pimicotinib in patients with tenosynovial giant cell tumor was started (NCT05804045).
Emactuzumab [1] (RG-7155) is a humanized monoclonal antibody directed against colony stimulating factor 1 receptor (CSF-1R) expressed on macrophages [2] [3] and has demonstrated a profound antitumor effect through interference with the CSF-1/CSF-1R axis, along with a manageable safety profile in patients with diffuse-type tenosynovial giant cell tumors (d-TGCT).
Malignancy in giant-cell tumor is uncommon and occurs in about 2% of all cases. However, if malignant degeneration does occur, it is likely to metastasize to the lungs. Giant-cell tumors are normally benign, [1] with unpredictable behavior. [2] It is a heterogeneous tumor composed of three different cell populations.
Villonodular synovitis is a type of synovial swelling.. Types include: Pigmented villonodular synovitis; Giant cell tumor of the tendon sheath; Though they have very different names, they have the same histology, and stain positive for CD68, HAM56, and vimentin. [1]
This page was last edited on 4 September 2021, at 16:03 (UTC).; Text is available under the Creative Commons Attribution-ShareAlike 4.0 License; additional terms may apply.
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