摘要目的 对外周神经母细胞性肿瘤(pNT)应用国际神经母细胞性肿瘤病理分类(INPC)标准进行分类,探讨此分类与pNT临床、病理特点的关系及其在预后评估中的价值.方法 收集上海儿童医学中心病理科2000年11月至2009年12月期间135例pNT手术标本、临床资料,光镜观察,应用INPC标准分类、国际神经母细胞瘤分期系统(INSS)分期,结合随访资料,分析影响患者生存时间的因素.结果 (1)按INPC分类标准,135例中神经母细胞瘤80例(59.2%),节细胞神经母细胞瘤混合型(GNBi)24例(17.8%),节细胞神经瘤17例(12.6%),节细胞神经母细胞瘤结节型(GNBn)14例(10.4%);组织分化良好型90例(66.7%),组织分化不良型45例(33.3%).(2)根据INSS分期135例中Ⅰ期22例(16.3%),Ⅱ期24例(17.8%),Ⅲ期34例(25.2%),Ⅳ期55例(40.7%).(3)135例患者,存活75例,死亡60例.患者平均生存时间与组织学分型、病理学分类及临床分期均相关(均P＜0.05).结论 INPC分类标准清晰、易操作,对评估预后能提供准确的病理依据.

abstractsObjective To study the clinicopathologic characteristics of peripheral neuroblastic tumors and to investigate the prognostic significance of International Neuroblastoma Pathology Classification (INPC). Methods One hundred and thirty-five cases of peripheral neuroblastic tumors encountered in Shanghai Children's Medical Center were enrolled into the study. All the cases were classified according to INPC and International Neuroblastoma Staging System (INSS). The follow-up data were analyzed. Results The consensus diagnoses of the 135 cases were as follows: 80 cases (59. 2% ) of neuroblastoma, 24 cases ( 17. 8% ) of ganglioneuroblastoma, intermixed, 17 cases ( 12. 6% ) of ganglioneuroma and 14 cases (10.4%) of ganglioneuroblastoma, nodular. The cases were subdivided into 2 subgroups: favorable histology (number =90, 66. 7% ) and unfavorable histology (number =45, 33.3% ). According to INSS,the number of cases in stages Ⅰ , Ⅱ , Ⅲ and Ⅳ was 22 ( 16. 3% ), 24 ( 17. 8% ), 34 ( 25.2% ) and 55 (40. 7% ), respectively. The survival of peripheral neuroblastic tumors correlated with histologic diagnosis,INPC and INSS ( P ＜ 0. 05 ). Conclusion Diagnostic categorization of peripheral neuroblastic tumors according to INPC is of prognostic value.