盆腔及骶尾部区域神经母细胞瘤临床特点及预后  

Clinical characteristics and prognosis of neuroblastoma in the pelvic and sacral regions

作　　者：刘甜 张诗晗 金眉[1] 张大伟[1] 秦红[2] 王焕民[2] 马晓莉[1] Liu Tian;Zhang Shihan;Jin Mei;Zhang Dawei;Qin Hong;Wang Huanmin;Ma Xiaoli(Department of Medical Oncology,Pediatric Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing Key Laboratory of Pediatric Hematology Oncology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China;Department of Oncology Surgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院儿童肿瘤中心肿瘤内科,北京市儿童血液肿瘤重点实验室,儿科重大疾病研究教育部重点实验室,北京100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院肿瘤外科,北京100045

出　　处：《中华实用儿科临床杂志》2021年第14期1091-1093,共3页Chinese Journal of Applied Clinical Pediatrics

基　　金：国家科技重大专项(2017ZX09304029);国家自然科学基金培育基金项目(GPY201703)。

摘　　要：目的总结原发部位在盆腔/骶尾部神经母细胞瘤(NB)患儿的临床特征、治疗效果并随访远期术后并发症。方法回顾性分析2007年3月至2019年6月在首都医科大学附属北京儿童医院血液肿瘤中心诊治的原发于盆腔或骶尾部NB患儿16例(男8例,女8例)的初诊年龄、原发瘤灶部位、瘤灶大小、临床分期、危险度分组等临床特征。对规律治疗随访的患儿进行临床特征分析,总结患儿术后并发症,并采用Kaplan-Meier方法进行生存分析。结果16例患儿中位诊断年龄23.0(5.7~102.0)月龄,6例(37.5%)≤12个月。患儿均进行了化疗,中位化疗疗程6(1~8)个疗程;15例患儿进行手术治疗,完全切除12例,占80%,手术方式以经腹为主(86.7%,13/16例)。患儿中位随访时间33.5(8~136)个月,3例(18.8%)患儿术后骶骨缺如,患儿术后均未出现永久性神经系统损伤。5年总生存(OS)率100%。结论单中心总结显示,盆腔和骶尾部区域NB患儿生存率高。肿瘤完全切除联合化疗治疗是有效的措施。术后永久性术后神经系统并发症少见。Objective To summarize the clinical characteristics,treatment response and long-term postoperative complications in children with neuroblastoma(NB)in the pelvic and sacral regions as the primary site.Methods The clinical characteristics of 16 NB children(8 males and 8 females)with primary pelvic and sacral admitted to the Department of Hematology Oncology Center in Beijing Children′s Hospital,Capital Medical University from March 2007 to June 2019 were analyzed retrospectively with respect to the age at first diagnosis,primary tumor site,tumor size,clinical stage,risk grouping,and other clinical characteristics.The clinical characteristics of the patients who were followed up for regular treatment were analyzed,and the postoperative complications of the patients were summarized,and the Kaplan-Meier method was used for survival analysis.Results The median age at diagnosis of these 16 children was 23.0 months(5.7-102.0 months),of which 6 cases(37.5%)were younger than 12 months old.All these children received chemotherapy,with a median of 6(1-8)courses of chemotherapy.Fifteen children received surgical resection of the pelvic tumor,with complete resection in 12 cases(80%).The surgical approach was mainly transabdominal(86.7%,13/16 cases).The median follow-up time of these children was 33.5(8-136)months.The patella was absent in 3 patients(18.8%)after the operation,and no permanent neurological damage occurred in all patients.Five-year overall survival(OS)rate was 100%.Conclusions A single-center summary showed a high survival rate for NB patients in the pelvic and sacral regions.Complete tumor resections combined with chemotherapy could be effective measures and rare cases occurred permanent postoperative neurological complications.

关 键 词：神经母细胞瘤 盆腔/骶尾 预后 

分 类 号：R739.4[医药卫生—肿瘤]