Abstract
BackgroundTenosynovial giant cell tumor (TGCT) is a rare, benign lesion affecting the synovial lining of joints, bursae, and tendon sheaths. It is generally characterized as a locally aggressive and often recurring tumor. A distinction is made between localized- and diffuse-type. The impact of TGCT on daily living is currently ill-described.ObjectiveThe aim of this crowdsourcing study was to evaluate the impact of TGCT on physical function, daily activities, societal participation (work, sports, and hobbies), and overall quality of life from a patient perspective. The secondary aim was to define risk factors for deteriorated outcome in TGCT.MethodsMembers of the largest known TGCT Facebook community, PVNS is Pants!!, were invited to an e-survey, partially consisting of validated questionnaires, for 6 months. To confirm disease presence and TGCT-type, patients were requested to share histological or radiological proof of TGCT. Unpaired t tests and chi-square tests were used to compare groups with and without proof and to define risk factors for deteriorated outcome.ResultsThree hundred thirty-seven questionnaires, originating from 30 countries, were completed. Median age at diagnosis was 33 (interquartile range [IQR]=25-42) years, majority was female (79.8% [269/337]), diffuse TGCT (70.3% [237/337]), and affected lower extremities (knee 70.9% [239/337] and hip 9.5% [32/337]). In 299 lower-extremity TGCT patients (32.4% [97/299]) with disease confirmation, recurrence rate was 36% and 69.5% in localized and diffuse type, respectively. For both types, pain and swelling decreased after treatment; in contrast, stiffness and range of motion worsened. Patients were limited in their employment (localized 13% [8/61]; diffuse 11.0% [21/191]) and sport-activities (localized 58% [40/69]; diffuse 63.9% [147/230]). Compared with general US population, all patients showed lower Patient-Reported Outcomes Measurements Information System-Physical Function (PROMIS-PF), Short Form-12 (SF-12), and EuroQoL 5 Dimensions 5 Levels (EQ5D-5L) scores, considered clinically relevant, according to estimated minimal important difference (MID). Diffuse versus localized type scored almost 0.5 standard deviation lower for PROMIS-PF (P<.001) and demonstrated a utility score of 5% lower for EQ-5D-5L (P=.03). In localized TGCT, recurrent disease and ≥2 surgeries negatively influenced scores of Visual Analog Scale (VAS)-pain/stiffness, SF-12, and EQ-5D-5L (P<.05). In diffuse type, recurrence resulted in lower score for VAS, PROMIS-PF, SF-12, and EQ-5D-5L (P<.05). In both types, patients with treatment ≤1year had significantly lower SF-12.ConclusionsTGCT has a major impact on daily living in a relatively young and working population. Patients with diffuse type, recurrent disease, and ≥2 surgeries represent lowest functional and quality of life outcomes. Physicians should be aware that TGCT patients frequently continue to experience declined health-related quality of life and physical function and often remain limited in daily life, even after treatment(s).
Highlights
Recurrence resulted in lower score for Visual Analog Scale (VAS), PROMIS-PF, Short Form-12 (SF-12), and EQ-5D-5L (P
Tenosynovial giant cell tumor (TGCT), previously pigmented villonodular synovitis (PVNS), is a rare, proliferative neoplasm affecting the synovial lining of joints, bursae, and tendons sheaths
Since younger patients are more likely to be on the World Wide Web, and our included patient population had a median age of 33 (25-42) years at time of diagnosis, in concordance with the WHO classification [1,2] and Mastboom et al [3], we considered our participants representative for the heterogeneous disease TGCT
Summary
Tenosynovial giant cell tumor (TGCT), previously pigmented villonodular synovitis (PVNS), is a rare, proliferative neoplasm affecting the synovial lining of joints, bursae, and tendons sheaths. Additional symptoms might be limited range of motion, instability, giving way, and locking complaints [4] Due to these unspecific signs and the rarity of the disease, patients frequently experience a delay of years in diagnosis [3,5,6]. To treat these symptoms, current treatment of choice is surgical excision, either by arthroscopic or open synovectomy [7]. Tenosynovial giant cell tumor (TGCT) is a rare, benign lesion affecting the synovial lining of joints, bursae, and tendon sheaths It is generally characterized as a locally aggressive and often recurring tumor. The impact of TGCT on daily living is currently ill-described
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