Popis: |
OBJECTIVE: Diffuse-type Tenosynovial Giant Cell Tumour (TGCT) is a rare, locally aggressive and difficult to treat disease. An international multicentre-pooled retrospective study of individual patient data was developed to describe global treatment protocols, evaluate oncological outcome, complications and functional results. A secondary study aim was to identify risk factors for local recurrence after surgical treatment. METHODS: Patients treated in 31 sarcoma reference centres between 1990 and 2017, with histologically proven diffuse-TGCT of large joints were included. Of 1192 cases of diffuse-TGCT, 58% were female with a median age 35 years. 64% affected the knee and in 54% primary treatment was one-staged open synovectomy. Risk factors were tested in a univariate analysis and significant factors subsequently included for multivariate analysis, with first local recurrence after surgical treatment in a tertiary centre as the primary outcome. RESULTS: At a median follow-up of 54 (95%CI 50-58) months, recurrent disease developed in 44% of all surgically treated cases, with local recurrence free survival (RFS) at 3, 5, 10 years of 62%, 55% and 40%, respectively. The strongest risk factor for recurrent disease was prior recurrence (HR 3.5 95%CI 2.8-4.4, p 35years), bone-involvement (present;absent), surgical technique (open;arthroscopic) nor tumour size ( |