透明细胞肉瘤是软组织肉瘤的一种极其罕见的耐药亚型。这项回顾性分析旨在通过评估转诊中心的缓解率、无进展生存期 (PFS) 和总生存期 (OS) 来阐明姑息化疗对 CCS 的疗效。对1997年至2023年在肉瘤科治疗的CCS患者的姑息治疗进行回顾性分析。使用RECIST标准评估治疗反应，并使用Kaplan-Meier法计算PFS和OS。该分析涵盖 23 名接受 CCS 化疗的患者，其中 11 名 (47.8%) 为男性。开始姑息治疗的中位年龄为 32 岁（范围 18-59 岁）。中位随访时间为 8.2 个月。 4 名患者因 M1 疾病转诊至我们中心，6 名患者接受围手术期化疗并在随访期间病情进展。在第一线中，14 名患者接受基于蒽环类的化疗（60.9%），5 名患者接受异环磷酰胺（HD-IFO）治疗，4 名患者接受其他方案。 1 名患者（4.3%）获得部分缓解（PR），12 名患者（52.2%）获得疾病稳定（SD）作为最佳缓解。第 1 线的中位 PFS 为 2.79 个月（95% CI：2.04-8.38），第 2 线为 1.76 个月（95% CI：0.72-6.97）。一线姑息化疗的中位 OS 为 8.2 个月（95% CI：6.2-14），二线姑息化疗的中位 OS 为 4.6 个月（95% CI：3.9-NA）。围手术期接受蒽环类药物预处理会使 M1 患者的中位 PFS 恶化。 CCS 中观察到对常规化疗的反应较差，表明需要对该适应症进行进一步的临床试验。

Clear cell sarcoma is an ultra-rare chemoresistant subtype of soft tissue sarcoma. This retrospective analysis aimed to clarify the efficacy of palliative chemotherapy in CCS by assessing response rates, progression-free survival (PFS), and overall survival (OS) at a referral center. A retrospective analysis of palliative treatment was conducted on patients with CCS treated at the sarcoma unit from 1997 to 2023. Treatment responses were assessed using RECIST criteria, and the Kaplan-Meier method was used to calculate PFS and OS. The analysis covered 23 CCS chemotherapy-treated patients with 11 (47.8%) men. The median age at the palliative treatment start was 32 years (range 18-59). The median follow-up was 8.2 months. Four patients were referred to our centre for M1 disease, and 6 received perioperative chemotherapy and progressed during follow-up. In the first line, 14 patients received anthracycline-based chemotherapy (60.9%), five were treated with ifosfamide (HD-IFO), and four received other regimens. One patient (4.3%) achieved partial response (PR), and 12 patients (52.2%) achieved stable disease (SD) as the best response. Median PFS in 1 line was 2.79 months (95% CI: 2.04-8.38), and 1.76 months (95% CI: 0.72-6.97) in the second line. The median OS from first-line palliative chemotherapy was 8.2 months (95% CI: 6.2-14), and the second-line palliative chemotherapy mOS was 4.6 months (95% CI: 3.9-NA). Perioperatively anthracycline-pretreated worsened patients' median PFS in the M1 setting. Poor responses to conventional chemotherapy were observed in CCS, indicating a need for further clinical trials in this indication.