AB1394 MANAGEMENT OF PATIENTS WITH TENOSYNOVIAL GIANT CELL TUMOUR/PIGMENTED VILLONODULAR SYNOVITIS IN THE ERA OF SYSTEMIC TARGETED THERAPY
Authors: M. Cochard, M. Chevalerias, D. Waast, V. Crenn, B. Le Goff
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Pigmented Villonodular Synovitis (PVNS) or Tenosynovial Giant Cell Tumours (TGCT) is a rare benign but locally aggressive synovial tumour that can impact joint function and quality of life. The emergence of systemic treatments targeting CSF-1 has changed patient management, particularly for diffuse-type TGCT (D-TGCT).. <h3>Objectives:</h3> The aim of this study was to describe the management strategy and follow-up of patients referred to Nantes University Hospital <h3>Methods:</h3> This was a single-center retrospective descriptive observational study. We included all new cases of diffuse-type (D-TGCT) or localized-type (L-TCGT) managed in our hospital between January 2010 and June 2023. Patient selection was performed using keywords in the hospital database. We included adult patients with TGCT involving large and medium-sized joints. The diagnosis was based on biopsy or typical findings on MRI. <h3>Results:</h3> A total of 105 patients (mean age 38±15 years), 63% female 37% male, 41% diffuse-type (D-TCGT), 59% localized-type (L-TCGT) were included. The most involved joint was the knee (75%), followed by the ankle (14%) and hip (7%). The most commonly reported symptoms were pain (80%), followed by joint effusion (32%). Patients were referred to the orthopaedic surgeon or rheumatologist in 72% and 28% of cases respectively. On these 61% were referred to our center for therapeutic management with a pre-established diagnosis, 24% needed additional diagnostic investigations (MRI or biopsy) and 15% came directly with non-specific symptoms. The diagnosis was most frequently made on MRI, with only 24% undergoing a biopsy prior to a therapeutic decision-making. The most common treatment strategy was surgical synovectomy (53%), systemic targeted therapy included tyrosine kinase inhibitors (4%) and joint injection (isotopic or triamcinolone) (4%), while wait and see treatment was a fairly common treatment strategy (37%). The proportion of TCGT undergoing surgery did not differ between D-TCGT and L-TCGT. For unoperated D-TCGT, wait and see treatment (32%) was preferred to medical treatment (16%). The median number of consultations during a median follow-up of 12 months was 2 (Q1-Q3=2) [0-11]. A significant number of patients were lost to follow-up regardless of treatment (29.5%) with no predictive factors identified. Thirteen patients (13%, 26% of D-TCGT) underwent a second therapeutic sequence because of recurrence of their TCGT. <h3>Conclusion:</h3> Our findings showed that systemic targeted therapies still have a limited role in our center compared with surgery which remains the first-line treatment in the management of TCGT patients. Wait and see was a fairly common strategy. We also found that a significant number of patients were lost to follow-up. Overall, these results highlight the need for a multidisciplinary approach to improve management and follow-up of TGCT patients. <h3>REFERENCES:</h3> <b>NIL.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>