软组织透明细胞肉瘤（CCS）是一种罕见的间质恶性肿瘤黑素细胞亚型。本研究的目的是调查与生存率增加相关的临床和治疗因素，按临床分期分层，以确定最佳治疗方案。该研究是一项回顾性分析，涉及 2016 年 7 月至 11 月间经组织学证实的 CCS 的 117 名患者2017 年，他们在日本骨和软组织肿瘤登记处入组。五年和十年生存率分别为 41%（95% 置信区间 (CI) 29 至 52）和 37%（95% CI 25 至 49） ）， 分别。多变量分析显示，肿瘤大小 > 10 cm (p = 0.006)、诊断时有淋巴结转移 (p < 0.001)、诊断时有远处转移 (p < 0.001)、且未接受手术原发肿瘤（p = 0.019）与较差的生存率独立相关。对于 N0M0 CCS (n = 68)，远处转移的发生是生存的独立预后因素（早期（< 12 个月），风险比 (HR) 116.78（95% CI 11.69 至 1,166.50）；p < 0.001；晚期（ > 12 个月），HR 14.79（95% CI 1.66 至 131.63）；p = 0.016）；新辅助/辅助化疗（p = 0.895）和/或放疗（p = 0.216）与生存率没有显着相关。局部复发的五年累积发生率为 19%（95% CI 8 至 35），肿瘤的大小与局部复发率的增加显着相关（p = 0.012）。对于 N1M0 CCS (n = 18)，原发肿瘤和淋巴结转移均接受手术的患者的死亡风险显着较低 (HR 0.03 (95% CI 0.00 至 0.56)；p = 0.020)。对于 M1 CCS (n = 31)，原发肿瘤的切除与更好的生存率独立相关（HR 0.26（95% CI 0.09 至 0.76）；p = 0.013）。不同类型的全身治疗之间的生存率没有显着差异（p = 0.523）。原发肿瘤和淋巴结的完全切除与CCS患者更好的生存率相关。全身治疗似乎提供的益处有限，表明迫切需要新型全身药物。© 2023 英国骨科编辑协会

Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment.The study was a retrospective analysis involving 117 patients with histologically confirmed CCS, between July 2016 and November 2017, who were enrolled in the Bone and Soft Tissue Tumour Registry in Japan.The five- and ten-year survival rates were 41% (95% confidence interval (CI) 29 to 52) and 37% (95% CI 25 to 49), respectively. On multivariable analysis, the size of the tumour of > 10 cm (p = 0.006), lymph node metastasis at the time of diagnosis (p < 0.001), distant metastases at the time of diagnosis (p < 0.001), and no surgery for the primary tumour (p = 0.019) were independently associated with a poor survival. For N0M0 CCS (n = 68), the development of distant metastases was an independent prognostic factor for survival (early (< 12 months), hazard ratio (HR) 116.78 (95% CI 11.69 to 1,166.50); p < 0.001; late (> 12 months), HR 14.79 (95% CI 1.66 to 131.63); p = 0.016); neoadjuvant/adjuvant chemotherapy (p = 0.895) and/or radiotherapy (p = 0.216) were not significantly associated with survival. The five-year cumulative incidence of local recurrence was 19% (95% CI 8 to 35) and the size of the tumour was significantly associated with an increased rate of local recurrence (p = 0.012). For N1M0 CCS (n = 18), the risk of mortality was significantly lower in patients who underwent surgery for both the primary tumour and lymph node metastases (HR 0.03 (95% CI 0.00 to 0.56); p = 0.020). For M1 CCS (n = 31), excision of the primary tumour was independently associated with better survival (HR 0.26 (95% CI 0.09 to 0.76); p = 0.013). There was no significant difference in survival between the different types of systemic treatment (p = 0.523).Complete excision of the primary tumour and lymph nodes is associated with a better survival in patients with CCS. Systemic treatment appears to provide limited benefits, demonstrating a pressing need for novel systemic agents.© 2023 The British Editorial Society of Bone & Joint Surgery.