11544 Background: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive tumor originating in the synovial lining of the joint, bursa, and tendon sheath. Since TGCT typically impacts individuals 20-50 years of age, pediatric TGCT is ultra-rare. However, pediatric patients are often not included in clinical trials or prospective data. Real-world data are needed to understand the impact of TGCT on pediatric patients’ quality of life and any differences between adults with TGCT. Methods: The TGCT Support Registry is an international, prospective registry initiated in 2022 by TGCT Support, a program of The Life Raft Group. The registry is the largest registry of patients with TGCT and records patient-reported demographic, pathologic, clinical information. This cross-sectional analysis presents data of patients ≤ 18 years of age at time of enrollment with Diffuse (D)-TGCT, Localized (L)-TGCT, or unknown subtype. Results: A total of 122 pediatric patients (9.5%) were included from a 1,278-patient registry (Table). Most patients were female (67.2%), had D-TGCT (73.0%), in the knee (83.6%), and a median age at diagnosis of 14.5 years. Symptoms reported were pain (94.3%), limited joint range of motion (89.3%), and swelling (84.4%). >50% of pediatric patients were initially misdiagnosed and were more likely to be misdiagnosed than adults (62.3% vs 49.9%, p < 0.01). 64.8% of pediatric patients were diagnosed by orthopedic surgeons, and 52.5% were diagnosed ≥1 years after symptom onset. Half of pediatric patients had joint aspirations to manage symptoms (47.5%) and non-steroidal anti-inflammatories were common (76.2%). Surgery was the predominant treatment modality and 61.5% reported that surgery occurred ≤ 3 months of diagnosis. Pediatric patients with D-TGCT underwent an average of 3.4 surgeries, compared to 1.8 surgeries for those with L-TGCT. 66.3% of pediatric patients with D-TGCT had ≥1 post-operative recurrence compared to 15.0% of L-TGCT pediatric patients. Most pediatric patients were referred to general orthopedic surgeons (80.3%), only a third consulted an oncology specialist, and 35.3% were also treated by pediatricians. Systemic therapies (i.e., imatinib, nilotinib, pexidartinib) were prescribed infrequently to pediatric patients with D-TGCT (17.2%). Pediatric patients reported that pain often interfered with daily activities and enjoyment of life. Conclusions: This real-world analysis highlights the significant disease burden of pediatric TGCT, as compared to adults, which severely affecting their quality of life. The reliance on surgical treatment and underuse of multidisciplinary care emphasizes the unmet need for provider education and treatment advancements tailored to this population. Greater efforts to develop systemic therapies specific to pediatrics are warranted to reduce recurrence rates and improve quality of life. Characteristics of patients stratified by subtype (localized, diffuse, or unknown), including sex, age, region, location of disease, misdiagnosis, duration from symptom onset to diagnosis, average surgeries, recurrences, and use of systemic therapies. Diffuse(n=89, 73.0%) Localized(n=20, 16.4%) Unknown(n=13, 10.6%) Total(N=122) Female sex, n (%) 59 (66.3) 14 (70.0) 9 (69.2) 82 (67.2) Median age at Diagnosis, years (range) 14 (3 - 17) 15 (4 - 17) 14.5 (7 - 15) 14.5 (3 - 17) Median age at Enrollment, years (range) 16 (4-18) 17 (6-18) 15 (10-18) 16 (4-18) Located in the US, n (%) 47 (52.8) 11 (55.0) 4 (30.8) 62 (50.8) Location of disease, n (%) Knee 76 (85.4) 16 (80.0) 10 (76.9) 102 (83.6) Hip 8 (9.0) 4 (20.0) 1 (7.7) 13 (10.7) Ankle 5 (5.6) 0 (0.0) 0 (0.0) 5 (4.1) Other a 0 (0.0) 0 (0.0) 2 (15.4) 2 (1.6) Misdiagnosis, n (%) 56 (62.9) 10 (50.0) 10 (76.9) 76 (62.3) Time from Symptom Onset to Diagnosis, n (%) <12 months 42 (47.1) 8 (40.0) 4 (30.8) 54 (44.3) 12–24 months 24 (27.0) 5 (25.0) 3 (23.1) 32 (26.2) 25–60 months 14 (15.7) 4 (20.0) 3 (23.1) 21 (17.2) >60 months 6 (6.7) 3 (15.0) 2 (15.4) 11 (9.0) Diagnosed during Surgery 3 (3.4) 0 (0.0) 1 (7.7) 4 (3.3) Average surgeries, (SD) Median 3.4 (2.8)2 1.8 (1.5)1 1 (1.2)1 2.9 (2.5)2 Systemic Therapy, Check all that apply Pexidartinib Imatinib Nilotinib 20 (22.5)7 (7.9)12 (13.5)1 (1.1) 0 (0.0) 0 (0.0) 0 (0.0) 0 (0.0) 1 (7.7)1 (7.7)0 (0.0)0 (0.0) 21 (17.2)8 (7.4)12 (9.8)1 (0.8) Local Recurrences, n (%) Yes 1 Recurrence ≥ 2 Recurrences 59 (66.3)19 (21.3)40 (44.9) 3 (15.0)2 (10.0)1 (5.0) 4 (3.8)1 (7.7)3 (23.1) 66 (54.1)22 (18.0)44 (36.1) No I have not had surgery I am unsure 30 (33.7)12 (13.5)8 (9.0) 17 (85.0)2 (10.0)4 (20.0) 9 (69.2)5 (38.5)1 (7.7) 56 (45.9)19 (15.6)13 (10.7)
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