儿童头颈部横纹肌肉瘤98例生存情况分析

Survival of head and neck rhabdomyosarcoma in children:a clinical analysis of 98 cases

目的分析儿童头颈部横纹肌肉瘤(RMS)的原发部位、治疗方法、疗效及生存情况。方法回顾性收集2005年2月至2017年9月北京同仁医院儿科就诊的原发于头颈部经病理确诊的98例RMS患儿的临床资料,根据国际横纹肌肉瘤组织的标准确定临床分期及危险度分组,采用χ2检验、Kaplan-Merier生存分析对临床资料进行统计分析,比较不同原发部位、不同危险度分组RMS患儿的生存情况。结果98例患儿中位年龄为70(2,161)个月,男53例(54.1%),女45例(45.9%)。原发部位位于非眶非脑膜者9例(9.2%),眼眶区43例(43.9%),脑膜旁肿瘤46例(46.9%);低危组27例(27.6%),中危组42例(42.9%),高危组29例(29.5%)。随访至2018年7月30日,中位随访时间42(9,149)个月,死亡37例,存活60例,1例手术后失访,总生存率61.9%(60/97),生存时间(92.3±6.9)个月。肿瘤原发部位位于脑膜旁的患儿生存率[47.8%(22/46)]低于眼眶[74.4%(32/43)]及非眶非脑膜(6/8),差异有统计学意义(χ^2=9.437,P=0.009)。高危组患儿生存率[51.7%(15/29)]低于中危组[57.1%(24/42)]和低危组[80.8%(21/26)],差异有统计学意义(χ^2=6.132,P=0.047)。结论头颈部横纹肌肉瘤预后较好,但脑膜旁、高危患儿远期预后明显下降,提高高危及脑膜旁RMS的预后将是提高头颈部RMS患儿预后的关键。

Objective To analysis the prognosis of head and neck rhabdomyosarcoma(RMS)in pediatrics.Methods The clinical data of 98 pediatric patients with head and neck RMS admitted from February 2005 to September 2017 were retrospectively analyzed.The clinical staging and risk grouping were performed according to international rhabdomyosarcoma study group criterion.The treatment included chemotherapy,radiotherapy and surgery accordingly.The association of prognosis with the disease characters,treatment strategy of 98 patients RMS was analyzed by Chi-square test and Kaplan-Merier analysis with SPSS 20.0 software.Results There were 53 boys(54.1%)and 45 girls(45.9%)with a median age of 70 months(2-161 months).There were 43 cases of orbit RMS(43.9%),46 cases of paramenigneal RMS(46.9%)and 9 cases of non-paramenigneal and non-orbit RMS(9.2%).Twenty seven patients were classified as low risk(27.6%),42 patients as medium risk(42.9%),and 29 patients as high risk(29.5%).The patients were followed up for a medium of 42 months(9-149 months),37 cases were dead,60 cases were survival,and 1 lost the follow-up.Kaplan-Merier survival analysis showed that the overall survival(OS)was 61.9%(60/97),and the average survival time was(92.3±6.9)months.Univariate analysis showed that the OS of patients with paramenigneal RMS was poorer than that of patients with orbit RMS,non-paramenigneal and non-orbit RMS.The OS of high risk RMS patients was poorer than that of intermediate and low risk patients(χ^2=6.132,P=0.047).Conclusions The prognosis of head and neck RMS in children is generally good,but the prognosis of paramenigneal RMS and high-risk RMS is significantly declined.The primary site of paramenigneal and high risk are independent risk factors for the prognosis of head and neck RMS in children.