摘要
目的探讨儿童视路胶质瘤(OPG)的治疗方法和临床疗效。方法回顾性分析2014年9月至2019年10月首都医科大学附属北京世纪坛医院儿科收治的OPG患儿(年龄≤16岁)的临床资料。患儿常规行肿瘤切除或活组织检查术,术后接受改良德国国际儿科肿瘤协会(SIOP)低级别胶质瘤(LGG)2004方案化疗。疗效评估指标包括影像学反应(以儿童神经肿瘤学应答评估量表为标准)和临床症状的改善情况。采用Kaplan-Meier法分析患儿的无进展生存率(PFS)和总生存率(OS)。通过log-rank检验比较组间的生存率。结果共纳入57例患儿,其中54例经手术切除肿瘤,2例行活组织检查术,1例予保守治疗。3例接受长春新碱+卡铂化疗,54例接受长春新碱+卡铂+依托泊苷化疗(或联合贝伐珠单抗、放疗或口服靶向药治疗)。截至末次随访,所有患儿均存活,其中位随访时间(范围)为3.5年(2.3~7.1年)。完全缓解16例,部分缓解33例,微缓解2例,疾病稳定4例,疾病进展2例。57例患儿治疗的中位起效时间为6.0个月,5年PFS和OS分别为(73.0±7.2)%和100.0%。确诊时存在肿瘤脊髓播散、未能手术切除肿瘤患儿的PFS分别低于无肿瘤脊髓播散、行手术切除的患儿(均P<0.05)。至末次随访,除性早熟外,57例患儿的临床症状均获得不同程度的改善,且化疗耐受性良好。结论对于OPG患儿,采用包括手术和术后化疗、放疗及应用靶向药物等的个体化治疗,能够获得较好的临床疗效,提高患儿的PFS。手术切除肿瘤和无肿瘤脊髓播散的患儿生存预后更佳。
Objective To explore the therapy and its clinical efficacy in children with optic pathway glioma(OPG).Methods The clinical data of children with OPG admitted to the Pediatrics Department of Beijing Shijitan Hospital,Capital Medical University from September 2014 to October 2019 were retrospectively analyzed.After routine tumor resection or biopsy,the children received chemotherapy with modified German International Society of Pediatric Oncology(SIOP)low-grade glioma(LGG)2004 regimen.Efficacy evaluation indicators include imaging response(based on the Response Assessment of Pediatric Neuro-Oncology,RAPNO)and improvement of clinical symptoms.Progression-free survival(PFS)and overall survival(OS)were analyzed by Kaplan-Meier method.Survival rates between groups were compared by the log-rank test.Results A total of 57 children were enrolled,of which 54 underwent tumor resection,2 underwent biopsy,and 1 underwent conservative treatment.Three patients received vincristine+carboplatin chemotherapy,and 54 patients received vincristine+carboplatin+etoposide chemotherapy(or combined with bevacizumab,radiotherapy or oral targeted drug therapy).All patients were alive at the last follow-up,and the median follow-up time(range)was 3.5 years(2.3-7.1 years).Complete response was achieved in 16 cases,partial response in 33 cases,minor response in 2 cases,stable disease in 4 cases,and disease progression in 2 cases.The median onset time of the 57 patients was 6.0 months,and the 5-year PFS and OS were 73.0%±7.2%and 100.0%,respectively.The PFS of children with spinal dissemination at the time of diagnosis and those without surgical resection was lower than those without spinal dissemination or surgical resection(both P<0.05).At the last follow-up,except for precocious puberty,the clinical symptoms of 57 children were improved to varying degrees,and chemotherapy was well tolerated.Conclusions For children with OPG,individualized treatment including surgery and postoperative chemotherapy,radiotherapy,and targeted drugs can achieve re
作者
杜淑旭
田永吉
张金
王圆
廖指仪
李苗
龚小军
孙艳玲
孙黎明
Du Shuxu;Tian Yongji;Zhang Jin;Wang Yuan;Liao Zhiyi;Li Miao;Gong Xiaojun;Sun Yanling;Sun Liming(Department of Pediatrics,Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China;Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University,Beijing 100070,China)
出处
《中华神经外科杂志》
CSCD
北大核心
2022年第6期555-560,共6页
Chinese Journal of Neurosurgery
基金
首都卫生发展科研专项项目(首发2020-2-2047)
首都医科大学附属北京世纪坛医院院基金(2021-C04)。
