Objective: To evaluate the long-term outcomes and prognostic factors of postoperative residual or recurrent fibrosarcoma in children. Methods: Clinical data of 26 patients continually admitted to Shanghai Children's Medical Center between April 2004 and February 2019 with postoperative residual or recurrent fibrosarcoma were analyzed retrospectively. All patients were treated with Shanghai Children's Medical Center-rhabdomyosarcoma-1999 (SCMC-RS-99) regimen and timely radical tumor resection. Before chemotherapy, according to the surgery and imaging examination, 26 patients were divided into 2 groups: postoperative residual group and postoperative recurrent group. Clinical features and long-term follow-up results of patients were summarized. Kaplan-Meier analysis was used to evaluate the overall survival (OS) and event-free survival (EFS) rates, Log-Rank test and Cox proportional hazards models were used for univariate and multivariate prognostic analysis of factors including age (<3 years or 3-18 years old), gender, primary tumor site, postoperative stage, disease status, ETS variant 6 (ETV6) gene and chemotherapy drugs. Results: Among 26 cases, 13 were male and 13 were female, 17 cases were in postoperative residual group and 9 cases were in postoperative recurrent group. Until the last follow-up at December 31, 2019, the median follow-up time was 73 months (ranged from 10 to 188 months).The 5-year OS and EFS rates were (86±7)% and (77±9)%. Univariate analysis showed that, the 5-year EFS rate of postoperative residual group was significantly higher than that of the postoperative recurrent group ((94±5)% vs.(63±16)%,χ(2)=5.106,P=0.024), the 5-year EFS rate of patients <3 years old was significantly higher than that of patients 3-18 years old ((94±5)% vs. (62±17)%, χ(2)=6.507, P=0.011). Gender (χ(2)=0.445), primary tumor site (χ(2)=0.258), postoperative stage (χ(2)=3.046), ETV6 gene (χ(2)=1.496), and whether doxorubicin-containing drugs in chemotherapy (χ(2)=1.692) did not exhibit significant impact on 5-EFS rate (all P>0.05). Age, postoperative stage and disease status were included in COX proportional risk model for multivariate analysis, which showed that age >3 years old (HR=8.95, 95%CI 0.73-109.50, P=0.086), stage Ⅲ-Ⅳ (HR=16.50, 95%CI 0.84-321.40, P=0.065) and postoperative recurrence (HR=10.60, 95%CI 0.84-134.30, P=0.068) had no significant impact on EFS rate. Conclusion: Children with postoperative residual or postoperative recurrent fibrosarcoma still had good remission rate and long-term survival, especially young children without recurrence have a significant survival advantage.
目的： 评价初次手术术后残留及单纯手术后复发纤维肉瘤患儿的远期疗效及预后相关因素。 方法： 回顾性研究，研究对象为2004年4月至2019年2月上海儿童医学中心收治的26例术后残留或复发纤维肉瘤患儿，接受上海儿童医学中心横纹肌肉瘤1999（SCMC-RS-99）方案及适时肿瘤根治性切除术，化疗前根据手术及影像学检查将疾病状态分为首诊术后残留组（简称残留组）、单纯手术后复发再治疗组（简称复发组），总结患儿临床特点及远期随访结果，采用Kaplan-Meier生存分析评估生存率，应用Log-Rank检验及COX比例风险模型对年龄（<3岁或3~18岁）、性别、肿瘤原发部位、术后分期、疾病状态、ETS变体6（ETV6）基因、化疗方案进行单因素及多因素预后分析。 结果： 26例纤维肉瘤患儿中男13例，女13例；残留组17例，复发组9例。随访至2019年12月31日，随访时间73（10~188）个月，5年总体生存（OS）率为（86±7）%，5年无事件生存（EFS）率为（77±9）%。单因素分析显示，残留组5年EFS优于复发组，差异有统计学意义[（94±5）%比（63±16）%，χ(2)=5.106，P=0.024]；<3岁的患儿5年EFS明显高于3~18岁患儿，差异有统计学意义[（94±5）%比（62±17）%，χ(2)=6.507，P=0.011]。性别（χ(2)=0.445）、肿瘤原发部位（χ(2)=0.258）、术后分期（χ(2)=3.046）、ETV6基因（χ(2)=1.496）、化疗中是否含阿霉素药物（χ(2)=1.692）与EFS均无明显相关性（P均>0.05）。将年龄、术后分期、疾病状态纳入COX比例风险模型进行分析发现年龄>3岁（HR=8.95，95%CI 0.73~109.50，P=0.086）、单纯手术后复发再治疗（HR=10.60，95%CI 0.84~134.30，P=0.068）、Ⅲ~Ⅳ期（HR=16.50，95%CI 0.84~321.40，P=0.065）对EFS影响差异无统计学意义。 结论： 首次术后残留或术后复发再治疗的纤维肉瘤患儿仍有较好的缓解率及长期生存，尤其小年龄非复发患儿具有明显的生存优势。.
Keywords: Child; Fibrosarcoma; Prognosis; Recurrence.