目的 探讨AVDC/ICE方案治疗儿童颅外恶性横纹肌样瘤(MRT)的疗效。方法 对2020年1月至2023年1月接受AVDC/ICE方案治疗的10例颅外MRT患儿进行回顾性分析。结果 10例颅外MRT患儿，男女发病比例为1∶1，中位发病年龄为24.5(1~138)月龄。3例患儿初诊时发生远处转移，最常见的转移部位是肺。初诊肿瘤平均大小为(9.87±5.42) cm。2例肾恶性横纹肌样瘤(MRTK)患儿术前出现肿瘤破溃。10例患儿明确病理后均接受AVDC/ICE方案化疗。截至末次随访时间，7例患儿处于完全缓解(CR)状态，1例患儿处于部分缓解(PR)状态，目前予贝伐珠单抗+索拉菲尼+环磷酰胺维持治疗中。2例肾外非中枢神经系统恶性横纹肌样瘤(EERT)患儿死于肿瘤转移。中位随访时间为14(5~28)个月，1年总体生存率(OS)为78.8%，1年无事件生存率(EFS)为68.6%。10例患儿治疗过程中均未发生与治疗相关的严重不良事件。结论 AVDC/ICE方案是治疗儿童颅外MRT安全有效的化疗选择。

Objective This study aims to assess the therapeutic effectiveness of the AVDC/ICE regimen for children diagnosed with extracranial malignant rhabdoid tumors (MRT). Methods A retrospective analysis was conducted on 10 pediatric cases of extracranial MRT treated with the AVDC/ICE from January 2020 to January 2023. Results Clinical data from 10 pediatric patients with extracranial MRT were analyzed. The gender distribution was balanced (1:1), with a median age at diagnosis of 24.5 months (range 1-138 months). Three patients had distant metastases at diagnosis, predominantly in the lungs. The average size of primary tumors at diagnosis was (9.87±5.42) cm. Two patients with malignant rhabdoid tumor of the kidney (MRTK) experienced tumor rupture before surgery. Following pathological confirmation, all patients received the AVDC/ICE chemotherapy regimen. At the last follow-up, seven patients achieved complete remission (CR), one patient achieved partial remission (PR) and was receiving maintenance therapy with bevacizumab, sorafenib, and cyclophosphamide. Two patients with extra-renal extracranial rhabdoid tumor (EERT) succumbed to metastasis. The median follow-up was 14 months (range 5-28 months), with a one-year overall survival (OS) rate of 78.8% and a one-year event-free survival (EFS) rate of 68.6%. No severe treatment-related adverse events were observed. Conclusion The AVDC/ICE regimen emerges as a safe and effective chemotherapy option for managing extracranial MRT in pediatric patients.