椎管受累的婴幼儿神经母细胞瘤的临床特点与治疗效果评价  

Clinical characteristics and therapeutic outcomes of infants and young neuroblastoma children with spinal cord compression

作　　者：牟晓 杨晓梅[1] 宋丽[1] 姬牧远 郭庆伟[1] 张开慧[1] 李府[1] MOU Xiao;YANG Xiaomei;SONG Li;JI Muyuan;GUO qingwei;ZHANG Kaihui;LI Fu(Department of Hematology and Oncology,Children′s Hospital Affiliated to Shandong University,Jinan 250022,China)

机构地区：[1]山东大学附属儿童医院/济南市儿童医院血液肿瘤科,济南250022

出　　处：《中国小儿血液与肿瘤杂志》2025年第1期35-38,共4页Journal of China Pediatric Blood and Cancer

摘　　要：目的回顾性分析年龄小于24个月、存在椎管受累的神经母细胞瘤(NB)患儿的临床特征、预后影响因素,总结治疗效果,监测、随访其长期健康问题,为下一步优化方案提供借鉴。方法收集2017年10月—2022年12月于本院确诊、年龄小于24个月的NB伴椎管受累的患儿的临床及随访资料,末次随访时间截至2023年11月31日。结果符合纳入标准的患儿共18例。男12例,女6例,平均年龄12.7(5.5-24.0)个月,中位随访时间54.6(6.0-78.0)个月,有10例(56%)存在脊髓损伤。治疗方面:7例(39%)首选手术治疗,11例(61%)首选化疗。至随访结束,2例(11%)因疾病进行性进展死亡,16例(89%)原发病达完全缓解或部分缓解,5年EFS及OS率为(88.2±7.8)%。6例(6/10)患儿遗留不同程度后遗症,包括运动功能障碍及括约肌功能障碍。结论存在椎管受累且有脊髓压迫表现NB患儿前期症状表现不明显,部分儿童就诊时病程时间长,前期经手术及化疗治疗后大多数患儿原发病预后良好,但容易遗留神经系统后遗症。Objective To retrospectively analyze the clinical characteristics and prognosis of neuroblastoma(NB)with spinal cord compression in children younger than 24 months,and perform a long-term follow up to monitor the health problems.Methods Clincal data of 18 children with neuroblastoma withspinal cord compression diagnosed at our hospital from October 2017 to December 2022 were included,clinical characteristics,treatment methods and the follow-up data were collected,last follow-up time was November 31,2023.Results 18 patients met the inclusion criteria.There were 12 male and 6 female patients,with a mean age of 12.7(5.5-24.0)months.The median follow-up time was 54.6(6.0-78.0)months.10 patients(56%)had spinal cord injury.By the end of follow-up,2 patients(11%)died due to progressive disease progression,and 16 patients(89%)achieved complete response or partial response.The 5-year EFS and OS rates were(88.2±7.8)%.6 patients(6/10)had different degrees of sequelae,including motor dysfunction and sphincter dysfunction.Conclusions Most of the patients with spinal cord compression with NB are infants,and the early symptoms are not obvious,resulting in a long course of disease for some children.After early surgery and chemotherapy treatment,most patients have a good prognosis of the primary disease,but prone to neurological sequelae.

关 键 词：神经母细胞瘤 椎管受累 儿童 

分 类 号：R739.4[医药卫生—肿瘤]