Objective: To evaluate the efficacy and influencing factors of surgery combined with neoadjuvant chemoradiotherapy in the treatment of children with non-orbital head and neck rhabdomyosarcoma (HNRMS). Methods: Information from 45 children diagnosed as non-orbital HNRMS and subjected to surgery combined with neoadjuvant chemoradiotherapy in Beijing Children's Hospital affiliated to Capital Medical University from August 2017 to July 2021 was analyzed. The patients included 25 males and 20 females, aged from 1 to 17 years old. The primary tumor site, pathological subtype, clinical stage, risk group, therapeutic regimen, resection range and outcome of all cases were also collected. The survival curves were made using the Kaplan-Meier method and the potential prognostic factors were investigated by Cox regression analysis. Results: Fifteen (33.3%) of 45 children achieved negative surgical margin under complete tumor resection. The postoperative pathological results showed that there were 20 cases of embryonic subtype, 19 cases of alveolar subtype and 6 cases of spindle sclerosis subtype. The postoperative follow-up time ranged from 4 to 71 months, with a median of 26 months. During the follow-up period, 13 children died, among whom brain metastasis was the most common cause of death, accounting for 7/13. The 3-year overall survival rate was 67.6%. Multivariate analysis showed that non-embryonic subtype (HR=6.26, 95%CI: 1.52-25.87, P=0.011) and failure to reach R0 resection (HR=9.37, 95%CI: 1.18-74.34, P=0.034) were independent risk factors affecting overall survival rate. Conclusion: Surgery combined with neoadjuvant chemoradiotherapy can offer a good efficacy for children with non-orbital HNRMS. Non-embryonic subtype and resection without negative operative microscopic margins are independent risk factors for poor prognosis, and brain metastasis is the main cause of death in these children.
目的： 分析手术联合新辅助放化疗治疗儿童非眼眶型头颈部横纹肌肉瘤（head and neck rhabdomyosarcoma，HNRMS）的疗效，并识别预后影响因素。 方法： 回顾性分析2017年8月至2021年7月于首都医科大学附属北京儿童医院诊断并接受手术联合新辅助放化疗的非眼眶型HNRMS患儿45例，其中男性25例，女性20例，年龄1~17岁。信息采集包括肿瘤的原发部位、病理分型、临床分期、危险度分组、治疗方案、手术切除范围以及预后情况。采用Kaplan-Meier法计算患儿的总体生存率并通过Cox回归分析可能影响预后的因素。 结果： 45例患儿术中达到肿瘤完全切除镜下切缘阴性者15例。术后病理结果显示胚胎型20例、腺泡型19例、梭形硬化型6例。术后随访时间为4~71个月不等，中位随访时间为26个月。随访期间共13例患儿死亡，其中脑转移为最常见死亡原因（7/13）。3年总体生存率为67.6%，多因素分析显示病理分型为非胚胎型（HR=6.26，95%CI：1.52~25.87，P=0.011）及手术切除范围无法达到R0（HR=9.37，95%CI：1.18~74.34，P=0.034）是影响总体生存率的独立危险因素。 结论： 手术联合新辅助放化疗治疗儿童非眼眶型HNRMS可获得较好疗效，非胚胎型病理分型及无法达到术中镜下切缘阴性是预后不佳的独立危险因素，脑转移是导致该类患儿死亡的主要原因。.