儿童头颈部横纹肌肉瘤疾病进展的危险因素及预后分析  

Risk factors for disease progression and prognosis of pediatric head and neck rhabdomyosarcoma

作　　者：文圆[1] 张伟令[1] 张谊[1] 胡慧敏[1] 李静[1] 黄东生[1] Wen Yuan;Zhang Weiling;Zhang Yi;Hu Huimin;Li Jing;Huang Dongsheng(Department of Pediatrics,Tongren Hospital,Capital Medical University,Beijing 100176,China)

机构地区：[1]首都医科大学附属北京同仁医院儿科,北京100176

出　　处：《中华实用儿科临床杂志》2021年第15期1152-1156,共5页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的探讨56例头颈部横纹肌肉瘤(HNRMS)患儿治疗期间疾病进展的危险因素,并分析不同疾病事件类型患儿的预后。方法选取2013年6月1日至2019年6月30日首都医科大学附属北京同仁医院儿科收治的56例HNRMS患儿,收集诊断、治疗、预后因素和随访结果等临床资料。按疾病事件类型分为无疾病进展组(NPD组)[包含无事件组(NE组)和疾病复发组(RD组)]和疾病进展组(PD组),比较NE组、RD组和PD组患儿的临床资料和预后。进一步采用单因素和多因素分析NPD组和PD组,探讨HNRMS患儿疾病进展的危险因素。结果56例患儿中位随访时间为31.8个月(3.5~74.6个月)。其中NE组患儿30例,RD组12例,PD组14例,其5年总体生存(OS)率分别为100.0%、82.5%和11.9%,差异有统计学意义(P<0.001)。与NE组相比,PD组患儿肿瘤最大径>5 cm(P=0.008)和无初始放疗(P=0.001)比例更高。与RD组相比,PD组患儿肿瘤最大径>5 cm的比例更高(P=0.002),术后病理分组(H=12.982,P=0.009)和危险度分组(H=18.679,P<0.001)的级别均更高。单因素分析显示,肿瘤最大径>5 cm、腺泡型病理类型、无初始放疗、术后病理分组和危险度分组的高级别是疾病进展的显著危险因素。多因素分析结果显示,肿瘤最大径>5 cm(OR=6.75,95%CI:1.02~44.78)、腺泡型病理类型(OR=7.10,95%CI:1.08~46.57)和无初始放疗(OR=15.26,95%CI:2.38~97.70)是疾病进展的独立危险因素。结论HNRMS发生疾病进展的患儿OS率明显较差,且预后与有复发和无事件的患儿明显不同。肿瘤最大径>5 cm、腺泡型病理类型和无初始放疗是疾病进展的独立危险因素。病情进展的患儿往往有较高级别的术后病理分组和危险度分组。Objective To explore the risk factors for disease progression and the prognosis with different disease events in a historical cohort of 56 pediatric patients with head and neck rhabdomyosarcoma(HNRMS).Methods From June 1,2013 to June 30,2019,56 pediatric patients diagnosed as HNRMS treated in the Department of Pedia-trics,Beijing Tongren Hospital,Capital Medical University were recruited in the present study.Clinical data,including general information about the diagnosis,treatment,prognostic factors,and follow-up results were collected.Patients were divided into non disease-progression(NPD)groups,including non-event(NE)group and disease-relapse(RD)group,and disease-progression(PD)group based on the disease event.Clinical data and the prognosis in NE group,RD group and PD group were compared.Risk factors for disease progression in pediatric patients with HNRMS were further analyzed by univariate and multivariate analyses of NPD group and PD group.Results The median follow-up time of all 56 patients was 31.8 months(3.5-74.6 months).There were 30,12,and 14 patients in NE group,RD group and PD group,respectively.The estimated 5-year overall survival rate was 100.0%,82.5%,and 11.9%in NE group,RD group and PD group,respectively,which was statistically significant(P<0.001).Compared with those of NE group,significantly higher rates of patients with a minimum tumor size of 5 cm(P=0.008)and non-initial radiation therapy(P=0.001)were detected in PD group.In addition,a significantly higher rate of patients with a minimum tumor size of 5 cm(P=0.002),and more advanced postoperative pathological stage(H=12.982,P=0.009)and risk of disease severity(H=18.679,P<0.001)were detected in PD group than those of RD group.The univariate analysis demonstrated that tumor size>5 cm,alveolar rhabdomyosarcoma,non-initial radiation therapy,advanced postoperative pathological stage and high risk of disease severity were significant risk factors for disease progression of HNRMS.The multivariate analysis result showed that tumor size>5 cm(OR=6.75,

关 键 词：横纹肌肉瘤 儿童 疾病进展 预后 危险因素 头颈部 

分 类 号：R739.91[医药卫生—肿瘤]