Abstract：
BACKGROUND: Diffuse-type tenosynovial giant cell tumor (D-TGCT) is a mono-articular, soft-tissue tumor. Although it can behave locally aggressively, D-TGCT is a non-malignant disease. This is the first study describing the natural course of D-TGCT and evaluating active surveillance as possible treatment strategy.
METHODS: This retrospective, multicenter study included therapy naïve patients with D-TGCT from eight sarcoma centers worldwide between 2000 and 2019. Patients initially managed by active surveillance following their first consultation were eligible. Data regarding the radiological and clinical course and subsequent treatments were collected.
RESULTS: Sixty-one patients with primary D-TGCT were initially managed by active surveillance. Fifty-nine patients had an MRI performed around first consultation: D-TGCT was located intra-articular in most patients (n = 56; 95 %) and extra-articular in 14 cases (24 %). At baseline, osteoarthritis was observed in 13 patients (22 %) on MRI. Most of the patients\' reported symptoms: pain (n = 43; 70 %), swelling (n = 33; 54 %). Eight patients (13 %) were asymptomatic. Follow-up data were available for 58 patients; the median follow-up was 28 months. Twenty-one patients (36 %) had radiological progression after 21 months (median). Eight of 45 patients (18 %) without osteoarthritis at baseline developed osteoarthritis during follow-up. Thirty-seven patients (64 %) did not clinically deteriorate during follow-up. Finally, eighteen patients (31 %) required a subsequent treatment.
CONCLUSIONS: Active surveillance can be considered adequate for selected therapy naïve D-TGCT patients. Although follow-up data was limited, almost two-thirds of the patients remained progression-free, and 69 % did not need treatment during the follow-up period. However, one-fifth of patients developed secondary osteoarthritis. Prospective studies on active surveillance are warranted.
METHODS: This retrospective, multicenter study included therapy naïve patients with D-TGCT from eight sarcoma centers worldwide between 2000 and 2019. Patients initially managed by active surveillance following their first consultation were eligible. Data regarding the radiological and clinical course and subsequent treatments were collected.
RESULTS: Sixty-one patients with primary D-TGCT were initially managed by active surveillance. Fifty-nine patients had an MRI performed around first consultation: D-TGCT was located intra-articular in most patients (n = 56; 95 %) and extra-articular in 14 cases (24 %). At baseline, osteoarthritis was observed in 13 patients (22 %) on MRI. Most of the patients\' reported symptoms: pain (n = 43; 70 %), swelling (n = 33; 54 %). Eight patients (13 %) were asymptomatic. Follow-up data were available for 58 patients; the median follow-up was 28 months. Twenty-one patients (36 %) had radiological progression after 21 months (median). Eight of 45 patients (18 %) without osteoarthritis at baseline developed osteoarthritis during follow-up. Thirty-seven patients (64 %) did not clinically deteriorate during follow-up. Finally, eighteen patients (31 %) required a subsequent treatment.
CONCLUSIONS: Active surveillance can be considered adequate for selected therapy naïve D-TGCT patients. Although follow-up data was limited, almost two-thirds of the patients remained progression-free, and 69 % did not need treatment during the follow-up period. However, one-fifth of patients developed secondary osteoarthritis. Prospective studies on active surveillance are warranted.
摘要：
背景：弥漫性腱鞘膜巨细胞瘤（D-TGCT）是一种单关节，软组织肿瘤.虽然它可以在当地表现得很积极，D-TGCT是非恶性疾病。这是第一项描述D-TGCT的自然过程并评估主动监测作为可能的治疗策略的研究。
方法：本回顾性研究，多中心研究包括2000年至2019年间来自全球8个肉瘤中心的D-TGCT初治患者。首次咨询后最初通过主动监测管理的患者符合资格。收集有关放射学和临床过程以及后续治疗的数据。
结果：61例原发性D-TGCT患者最初接受主动监测。59例患者在首次咨询前后进行了MRI检查：大多数患者（n=56；95％）位于关节内，14例（24％）位于关节外。在基线,13例患者（22％）在MRI上观察到骨关节炎。大多数患者报告的症状：疼痛（n=43;70％），肿胀(n=33；54%)。8例患者（13％）无症状。58例患者获得了随访数据；中位随访时间为28个月。21名患者（36％）在21个月后出现放射学进展（中位数）。基线时没有骨关节炎的45例患者中有8例（18％）在随访期间发展为骨关节炎。37名患者（64％）在随访期间没有临床恶化。最后,18名患者（31%）需要后续治疗。
结论：主动监测可被认为足以适用于选择治疗的初治D-TGCT患者。尽管随访数据有限，几乎三分之二的患者仍然没有进展，69%的患者在随访期间不需要治疗.然而,五分之一的患者出现继发性骨关节炎.积极监测的前瞻性研究是必要的。
方法：本回顾性研究，多中心研究包括2000年至2019年间来自全球8个肉瘤中心的D-TGCT初治患者。首次咨询后最初通过主动监测管理的患者符合资格。收集有关放射学和临床过程以及后续治疗的数据。
结果：61例原发性D-TGCT患者最初接受主动监测。59例患者在首次咨询前后进行了MRI检查：大多数患者（n=56；95％）位于关节内，14例（24％）位于关节外。在基线,13例患者（22％）在MRI上观察到骨关节炎。大多数患者报告的症状：疼痛（n=43;70％），肿胀(n=33；54%)。8例患者（13％）无症状。58例患者获得了随访数据；中位随访时间为28个月。21名患者（36％）在21个月后出现放射学进展（中位数）。基线时没有骨关节炎的45例患者中有8例（18％）在随访期间发展为骨关节炎。37名患者（64％）在随访期间没有临床恶化。最后,18名患者（31%）需要后续治疗。
结论：主动监测可被认为足以适用于选择治疗的初治D-TGCT患者。尽管随访数据有限，几乎三分之二的患者仍然没有进展，69%的患者在随访期间不需要治疗.然而,五分之一的患者出现继发性骨关节炎.积极监测的前瞻性研究是必要的。
