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Objective: Adequate documentation of the outcome of treatment of pigmented villonodular synovitis (PVNS) is sparse. Available case series show relatively short follow-up times and often combine locations or subtypes to increase patient numbers. This article describes the long-term follow-up of a single institution's large consecutive series of PVNS., Methods: Retrospectively, 107 PVNS patients were identified between 1985 and 2011 by searching pathology and radiology records. Treatment complications, recurrences and quality of life were evaluated. Most patients (85.2%) were primarily or secondarily treated at our institution., Results: Both subtypes, localized PVNS [29 (27%)] and diffuse PVNS [75 (70%)] were represented. The knee was affected in 88% of patients. Treatments received were surgery, external beam radiotherapy, radiosynovectomy, targeted therapy, immunotherapy or combinations of these. Forty-nine (46%) patients had prior treatment elsewhere. The mean follow-up from diagnosis until last contact was 7.0 years (range 0.3-27.4) for localized PVNS and 14.5 years (range 1.1-48.7) for diffuse PVNS. The 1- and 5-year recurrence-free survival rates for diffuse PVNS were 69% and 32%, respectively. Quality of life, estimated by 36-item Short Form Health Survey (SF-36) scores, were not significantly different between localized and diffuse PVNS. However, both patient groups scored lower than the general population norms on the general health component (59.2 and 56.3, respectively, P < 0.05)., Conclusion: Recurrence rates of PVNS increase with time. Long-term follow-up shows, particularly in diffuse PVNS, it is a continually recurring problem, and over time it becomes increasingly difficult to cure. The quality of life is decreased in patients with PVNS compared with the general population., (© The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)