Giant cell tumor of the tendon sheath (GCTTS), tenosynovial giant cell tumor (TGCT), and pigmented villo-nodular synovitis (PVNS) are the common names for a group of rare proliferative disorders that involve synovial joints and tendon sheaths. Considerable confusion exists about the surgical treatment and diagnosis of these disorders. This review evaluates the presentation, surgical therapy and recurrence of these three proliferative disorders. We retrospectively reviewed the clinical data from all cases of GCTTS, TGCT, and PVNS from 1985 to 1997. A total of 35 patients were identified: GCTTS (n=8), TGCT (n=1), and PVNS (n=26), there were 18 men and 17 women. The median age was 48 years (range 6-84 years). The most common site of involvement was the knee (15), followed by wrist (7), elbow (4), and hip (4). Seven patients had extra-articular involvement, and 19 were found incidentally at operations for other reasons. Among the 4 patients who developed recurrent disease, 2 had extra-articular disease at the time of their original diagnosis. None died, and none required major amputation. One patient was treated with adjuvant radiotherapy following resection of a recurrence. It is important to distinguish between focal and diffuse forms of synovial involvement. If focal, simple surgical excision is appropriate. If diffuse, complete synovectomy is indicated for disease confined to the joint, and resection of all gross disease is indicated for extra-articular disease. Radical resection with negative margins is not necessary in most instances. In rare aggressive cases, local recurrence may necessitate more extensive resection and radiation therapy.

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