摘要目的 探讨外周神经母细胞性肿瘤(pNT)的临床、病理特点,分析这些因素在判断肿瘤预后中的作用.方法 收集121例儿童pNT的临床、病理及随访资料,依据神经母细胞瘤病理国际分类(INPC),对年龄、临床分期、病理学分型、核分裂-核碎裂指数(MKI)进行单因素预后分析.结果 (1)121例患儿,中位年龄2.7岁,年龄＜5岁者96例(79.3％).(2)神经母细胞瘤(NB)82例；混杂型节细胞性神经母细胞瘤(GNBi)9例；节细胞神经瘤(GN) 19例,均为即将成熟型；结节型节细胞性神经母细胞瘤(GNBn) 11例.预后良好的组织学类型(FH)49例,预后不良的组织学类型(UH)72例.(3)121例pNT患儿3年无瘤生存率为(73.0±4.3)％.3年无瘤生存率与患儿年龄(P=0.002)、Evan分期(P =0.000)、病理学分型(P=0.000)、MKI(P=0.043)及组织学预后分组(P =0.000)相关.结论 (1)pNT多见于5岁以下儿童,它包括NB、GNBi、GN及GNBn 4种类型.(2)pNT 3年无瘤生存率约70％,年龄、临床分期、病理学分型及MKI均是判断其预后的指标,INPC是切实可行的分类方法.

abstractsObjective To study the clinicopathologic characteristics of peripheral neuroblastic tumors and to evaluate the prognostic significance of these features.Methods The clinical and pathologic findings were retrospectively reviewed in 121 cases of peripheral neuroblastic tumor.The clinical outcomes of patients were evaluated.The three-year event-free survival rate was analyzed,with respect to age of patients,Evan's staging,International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.Results The median age at diagnosis was 2.7 years; and 96 cases (79.3％) occurred in patients younger than 5 years old.The number of cases in Evan's staging Ⅰ,Ⅱ,Ⅲ,Ⅳ and Ⅳ s was 24,39,24,29 and 5,respectively.There were 82 cases of neuroblastoma (NB) (including 2 cases of undifferentiated NB,52 cases of poorly differentiated NB and 28 cases of differentiating NB),9 cases of ganglioneuroblastoma,intermixed type (GNBi),19 cases of ganglioneuroma,maturing type (GN) and 11 cases of ganglioneuroblastoma,nodular type (GNBn).Forty-nine cases were in the favorable histology subgroup and 72 cases in the unfavorable histology subgroup.The overall three-year event-free survival rate of the 121 cases was 73.0％ ± 4.3％.The three-year event-free survival rates were associated with age (P =0.002),Evan's staging (P =0.000),histologic category (P =0.000),mitosis-karyorrhexis index (P =0.043),prognostic subgroup (P =0.000).Conclusions Most of the peripheral neuroblastic tumors occur in the children younger than 5 years old.It is composed of NB,GNBi,GN and GNBn.The three-year event-free survival rate is approximately 70％.Significant prognostic parameters include age of patients,Evan's staging,International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.