摘要
目的分析伴骨骼转移高危神经母细胞瘤(HR-NB)患儿的临床特点和相关预后因素。方法回顾性分析2007年1月至2018年12月首都医科大学附属北京儿童医院肿瘤内科收治的336例初诊伴骨骼转移HR-NB患儿临床资料,采用Kaplan-Meier法进行生存分析,单因素预后分析采用Log-Rank检验,Cox模型用于多因素预后分析。结果336例患儿发病中位年龄43(4~148)月龄;男188例,女148例。面颅骨转移89例(26.5%),脑颅骨转移193例(57.4%),椎骨转移298例(88.7%),胸肋骨转移183例(54.5%),骨盆转移270例(80.4%),上肢骨182例(54.2%)和下肢骨转移240例(71.4%)。患儿5年无事件生存(EFS)率和总生存(OS)率分别为(30.4±2.7)%和(41.3±2.9)%。单因素分析显示,预后分类不良、形态学为神经母细胞瘤间质贫乏和节细胞神经母细胞瘤混合型、有丝分裂核分裂指数高、乳酸脱氢酶≥587 U/L、血清铁蛋白≥92μg/L、MYCN扩增及1p杂合性缺失及面颅骨、脑颅骨、胸肋骨、骨盆、上肢骨和下肢骨骨骼转移的HR-NB患儿5年OS率显著降低(P<0.05)。全部7部位广泛骨骼转移患儿5年OS率仅为(14.2±5.9)%,显著低于单部位及其他多部位骨骼转移患儿[5年OS率分别为(66.0±10.2)%和(43.6±3.4)%,χ^(2)=45.722,P<0.05]。Cox多因素分析显示,MYCN扩增和面颅骨骨骼转移是影响伴骨骼转移HR-NB患儿预后的独立危险因素(HR=4.165、2.560,95%CI:2.356~7.363、1.519~4.315,均P<0.05)。结论初诊时伴多部位广泛骨骼转移HR-NB患儿预后极差。MYCN扩增和面颅骨骨骼转移是影响伴骨骼转移HR-NB患儿预后的不良因素。
Objective To analyze the clinical characteristics and prognostic factors of high-risk neuroblastoma(HR-NB)patients with skeletal metastasis.Methods The clinical features of 336 newly treated HR-NB patients with skeletal metastases admitted to the Department of Medical Oncology of Beijing Children′s Hospital,Capital Medical University from January 2007 to December 2018 were analyzed retrospectively.Kaplan-Meier method was used for the survival analysis,and Log-Rank test was used for univariate prognosis analysis.The Cox model was used to analyze the multifactorial prognostic analysis.Results A total of 336 HR-NB patients were recruited,involving 188 males and 148 females with the median age of onset of at 43(4-148)months.Skeletal metastases affected the viscerocranium(89 cases,26.5%),neurocranium(193 cases,57.4%),vertebrae(298 cases,88.7%),sternum and ribs(183 cases,54.5%),pelvis(270 cases,80.4%),upper limbs(182 cases,54.2%)and lower limbs(240 cases,71.4%).The 5-year event-free survival(EFS)rate and overall survival(OS)rate were(30.4±2.7)%and(41.3±2.9)%,respectively.Univariate analysis showed a significantly lower 5-year OS rate in skeletal metastatic HR-NB patients with poor prognostic classification,the morphology of neuroblastoma(stroma-poor)and ganglioneuroblastoma(intermixed),high index of mitosis-karyorrhexis index,lactate dehydrogenase≥587 U/L,serum ferritin≥92μg/L,MYCN amplification and 1p loss of heterozygosity,and metastases in the viscerocranium,neurocranium,vertebrae,sternum and ribs,pelvis,upper limbs and lower limbs(all P<0.05).The 5-year OS rate of HR-NB patients with all 7 regions of skeletal metastases was only(14.2±5.9)%,which was significantly lower than that in patients with a single region metastasis or multi-region metastases[(66.0±10.2)%vs.(43.6±3.4)%,χ^(2)=45.722,P<0.05].Cox multifactorial analysis showed that MYCN amplification(HR=4.165,95%CI:2.356-7.363)and the viscerocranium metastasis(HR=2.560,95%CI:1.519-4.315)were the independent risk factors affecting the prognosis of HR
作者
范洪君
黄程
周宇晨
朱帅
段超
赵文
赵倩
王希思
张大伟
金眉
马晓莉
苏雁
Fan Hongjun;Huang Cheng;Zhou Yuchen;Zhu Shuai;Duan Chao;Zhao Wen;Zhao Qian;Wang Xisi;Zhang Dawei;Jin Mei;Ma Xiaoli;Su Yan(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)
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2023年第5期331-338,共8页
Chinese Journal of Applied Clinical Pediatrics
基金
北京市自然科学基金(7222054)。
关键词
神经母细胞瘤
高危
骨骼转移
预后
Neuroblastoma
High risk
Skeletal metastasis
Prognosis
