Abstract：
Our study was carried out to define the efficacy of treatment with sequential chemotherapy lines in patients with epithelioid sarcoma (ES) at referral centres for sarcoma. From 1998 to 2023, 22 patients with ES were treated with chemotherapy and included in the analysis. The median age at the start of palliative treatment was 35 (20-68). The median follow-up was 22.1 months. In the first line, 13 patients (59%) received anthracycline-based chemotherapy and 6 (27%) high-dose ifosfamide. One patient (4.5%) achieved PR, 15 (68%) SD, and 6 (32%) PD as the best response. The median progression-free survival (PFS) in the first line was 6.4 months (95% CI: 3.02-12.9), but 9.7 months (95% CI: 4.37-NR) for chemotherapy based on anthracycline, indicating a more favourable PFS (p = 0.027). Twenty (90%) patients received second-line treatment, and eleven received third-line chemotherapy. The median OS from the start of first-line palliative chemotherapy was 22.1 months (95% CI: 10.5-41.4) and 14.7 months from the beginning of the second line. Perioperatively, patients pretreated with anthracycline had a median PFS of 2.9 months in the M1 setting. Second-line long-time responses were achieved with pazopanib or vincristine with actinomycin D. Despite chemoresistance, an advantage associated with anthracycline-based chemotherapy was confirmed in the ES cohort. Poor responses underscore the need for further research on targeted therapies for ES. Second-line chemotherapy or clinical trials should be offered to all eligible patients.
摘要：
我们的研究是为了确定在肉瘤转诊中心对上皮样肉瘤（ES）患者进行序贯化疗的疗效。从1998年到2023年，22例ES患者接受了化疗并纳入分析。姑息治疗开始时的中位年龄为35岁（20-68岁）。中位随访时间为22.1个月。在第一行中，13例患者（59％）接受蒽环类化疗和6例（27％）大剂量异环磷酰胺。一名患者（4.5%）达到PR，15（68%）SD，和6(32%)PD为最好反响。一线中位无进展生存期(PFS)为6.4个月(95%CI:3.02-12.9),但以蒽环类药物为基础的化疗为9.7个月（95%CI：4.37-NR），表明PFS更有利(p=0.027)。20名(90%)患者接受二线治疗,11人接受三线化疗。从一线姑息性化疗开始的中位OS为22.1个月（95％CI：10.5-41.4）和从二线开始的14.7个月。围手术期,在M1组,接受蒽环类药物预处理的患者的中位PFS为2.9个月.帕唑帕尼或长春新碱与放线菌素D实现了二线长期反应。在ES队列中证实了以蒽环类为基础的化疗的优势.反应不佳强调需要进一步研究ES的靶向疗法。应向所有符合条件的患者提供二线化疗或临床试验。
