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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).
The decision comes months after independent experts on an advisory panel to the FDA voted in favor of Turalio as a treatment for the debilitating condition called tenosynovial giant cell tumor (TGCT).
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.
Definitive diagnosis is made by tumor biopsy. [12] Surgery is the most common method of treating peripheral nerve sheath tumors. [11] In malignant tumors, complete resection is the only known curative treatment (with a sufficiently wide margin or even amputation to improve prognosis). [12]
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).
Giant-cell tumor of the tendon sheath, also known as giant-cell synovioma, localized nodular tenosynovitis and localized tenosynovial giant cell tumor or TGCT [1] is a firm lesion, measuring 1 to 3 cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces.
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.
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