高危头颈部横纹肌肉瘤临床特征及预后分析

Study on the clinical characteristics and prognosis for children high-risk group of head and neck rhabdomyosarcoma

目的研究高危头颈部横纹肌肉瘤(RMS)的高危因素与诊疗及预后的相关性。方法根据美国儿童肿瘤组织(COG)、中国抗癌协会小儿血液肿瘤学组儿童横纹肌肉瘤协作组(CCCG-RMS)制定的危险度分组,筛选原发于头颈部的高危儿童横纹肌肉瘤为研究对象。采用穿刺病理/手术+化疗+放疗等综合治疗,分析原发部位、年龄、病理分型、转移等因素与诊疗策略、预后的相关性。结果入组患儿29例。其中男17例(58.6%),女12例(41.4%);原发部位:眼眶区占位7例(24.1%),脑膜旁占位22例(75.9%);临床分期:Ⅲ期2例(6.9%),Ⅳ期27例(93.1%)。随访至2018年12月31日,死亡14例,存活15例,无病生存10例,总生存率为48.3%(15/29例),无病生存率为34.5%(10/29例)。Kaplan-Merier曲线分析提示总生存时间(76.0±12.0)个月,95%可信区间(CI):53.5~93.2个月;无事件生存时间(62.5±10.6)个月,95%CI:47.0~83.9个月。结论高危组头颈部RMS患儿预后相对较差,中枢神经侵犯是重要的致死因素,密切随访及防治中枢神经系统侵犯是提高RMS预后的主要任务。

Objective To study the relationship between high-risk factors with the diagnosis,treatment and prognosis of children with high-risk head and neck rhabdomyosarcoma(RMS).Methods Children with high-risk RMS primarily found in head and neck were selected as research objects according to the criteria of RMS risk degree formulated by Children Oncology Group(COG)and Children′s Rhabdomyosarcoma Cooperative Group,Children′s Hematological Oncology Group,China Anti-Cancer Association(CCCG-RMS),and combined treatment including puncture pathology/surgery,chemotherapy and radiotherapy were performed.The relationship with primary site,age,pathology type and metastasis and prognosis were analyzed.Results Twenty-nine patients were collected as research object,17 cases(58.6%)of male,and 12 cases(41.4%)of female.The primary site:orbit was 7 patients(24.1%),and para-meningeal was 22 patients(75.9%).Clinical stage:2 cases were stageⅢ(6.9%),and 27 cases were stageⅣ(93.1%).Followed up to December 31,2018,14 patients were dead,15 patients were survival,and 10 patients were event free survival.The overall survival rate was 48.3%(15/29 cases),and the event free survival rate was 34.5%(10/29 cases).According to Kaplan-Merier survival curve analysis,the average overall survival time was(76.0±12.0)months,95%confidence interval(95%CI):(53.5-93.2)months.The event free survival time was(62.5±10.6)months,95%CI:(47.0-83.9)months.Conclusions The prognosis of high-risk head and neck RMS is poor.The center system metastasis is cause of death.Thus,the main task is how to close follow-up and prevention of central nervous system invasion for improving prognosis.