婴儿神经母细胞瘤51例诊疗特点及长期随访分析  

Diagnosis and treatment characteristics and long-term follow-up of 51 cases of infantile neuroblastoma

作　　者：徐权 袁晓军[1] XU Quan;YUAN Xiaojun(Pediatric Hematology and Oncology,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China;Department of Pediatrics,Putuo Hospital Affiliated to Shanghai University of Traditional Chinese Medicine,Shanghai 200062,China)

机构地区：[1]上海交通大学医学院附属新华医院儿血液肿瘤科,上海200092 [2]上海中医药大学附属普陀医院儿科,上海200062

出　　处：《临床儿科杂志》2023年第9期680-685,共6页Journal of Clinical Pediatrics

摘　　要：目的分析初诊年龄<12月龄神经母细胞瘤(NB)患儿的临床特征、诊疗特点及远期疗效。方法回顾性分析2008年1月至2018年12月收治的NB患儿的临床资料,总结其临床特点及预后影响因素。结果51例NB患儿中,男34例、女17例,中位诊断年龄7.5(3.8~10.1)月龄,腹部肿块(27例,52.9%)是最常见的就诊原因。原发部位主要为肾上腺(21例,41.1%)及后腹膜(19例,37.2%),骨髓、肝脏及骨是最常见的转移部位。MYCN基因扩增频率为11.3%(5/44)。49例患儿行手术治疗,其中8例单纯手术治疗,13例化疗后行手术治疗,28例化疗前行手术治疗;1例仅行化疗,1例随访观察。中位随访时间为78.5(72.1~124.0)月龄,共49例无事件生存,2例死亡。6年总体生存率和6年无事件生存率均为(96.1±2.7)%。单因素分析提示肿瘤分期、远处转移、骨髓转移、骨转移、危险度分组、MYCN基因扩增、初诊时血清神经元特异性烯醇化酶和乳酸脱氢酶水平是影响预后的因素(P<0.05)。结论婴儿NB患者长期预后好,无MYCN基因扩增的患儿有望进一步降低化疗强度。Objective The clinical characteristics,diagnosis and treatment characteristics and long-term curative effect of the children with neuroblastoma(NB)at the age of less than 12 months were analyzed to provide evidencebased basis for further optimizing the treatment of infantile NB patients.Methods The clinical data of infantile NB patients admitted from January 2008 to December 2018 were retrospectively analyzed,and the clinical characteristics and prognostic factors of the children were summarized.Results The median age of diagnosis in 51 NB infants(34 boys and 17 girls)was 7.5(3.8-10.1)months,and abdominal mass(27 cases,52.9%)was the commonest reason for medical treatment.The most frequently-occurring sites of tumors were adrenal gland(21 cases,41.4%)and retroperitoneum(19 cases,37.2%).Bone marrow,liver and bone were the commonest sites of metastasis.The amplification frequency of MYCN gene was 11.3%(5/44).Forty-nine NB patients underwent surgery,of whom 8 were treated with surgical resection alone,13 were treated with surgery after chemotherapy,and 28 were treated with surgery before chemotherapy.Of the remaining patients,1 received chemotherapy alone and 1 did not receive any treatment except follow-up.The median follow-up time was 78.5(72.1-124.0)months.A total of 49 patients survived without an event.Two patients died.The 6-year overall survival rate and the 6-year event-free survival rate were both(96.1±2.7)%.Univariate analysis revealed that tumor staging,distant metastasis,bone marrow metastasis,bone involvement,risk grouping,MYCN amplification,serum neuron-specific enolase and lactate dehydrogenase levels at initial diagnosis were factors affecting prognosis(P<0.05).Conclusions Infantile NB patients have a good long-term prognosis.It should be expected to further reduce the intensity of chemotherapy for patients without MYCN amplification.

关 键 词：神经母细胞瘤 临床特点 预后 婴儿 

分 类 号：R739.4[医药卫生—肿瘤]