Tenosynovial giant cell tumor

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Synonyms: Diffuse-type GCT | Diffuse-type giant cell tumor | Pigmented villonodular synovitis | TGCT | TSGCT

A rare benign proliferative disorder of the synovial membrane primarily affecting young adults (with a peak age of onset in the second to fourth decade of life) characterized by proliferative locally invasive tumor-like lesions usually involving a single joint tendon sheath or bursa (most commonly the joints of the knee and hip and rarely others such as the ankle shoulder and temporomandibular joints). It presents with pain and limitation of motion along with swelling heat and tenderness over the involved joint eventually leading to arthritic degeneration and significant locomotor deficit if left untreated. PVNS can recur in patients even after treatment.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version June 2024

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