摘要
目的探讨小儿神经母细胞肿瘤（NT）MYCN基因扩增的意义。方法收集154例NT患儿的临床病理资料，包括患儿一般资料、病理类型、临床分期及预后等。应用荧光原位杂交（FISH）检测MYCN基因扩增情况，分析MYCN扩增与临床病理特征及预后的关系。结果154例NT患儿年龄1d-11岁，平均26．1个月，中位数为20．5个月；男女比例为1．48：1．00；临床分期Ⅰ期20例（13．0％），Ⅱ期23例（14．9％），Ⅲ期43例（27．9％），Ⅳ期64例（41．6％），Ⅳs期4例（2．6％）；组织学预后良好型72例（46．8％），组织学预后不良82型（53．2％）。20例MYCN扩增（13．O％），MYCN与2号染色体（CEP2）信号比值为4．08～43．29；MYCN无扩增134例，其中获得91例（68．0％），阴性43例（32．0％）。MYCN的表达在不同年龄、神经母细胞瘤分型、组织学预后分组以及核分裂-核碎屑指数（MKI）高低及临床分期差异均有统计学意义（P均〈0．05），而与性别无关（P〉0．05）。20例MYCN扩增病例中4例存活（20．0％），16例死亡（80．O％），生存期为（17．10±2．24）个月。MYCN无扩增134例中96例存活（71．6％），38例死亡（28．4％），生存期（28．71±1．28）个月。生存分析结果显示，MYCN扩增患儿预后较差（Х^2=19．596，P〈0．05）。结论MYCN基因扩增患儿预后差，我国儿童NT扩增率较低。
Objective To explore the significance of MYCN gene amplification in children with neuroblastic tumors （NT）. Methods The clinicopathological data of 154 cases with NT were reviewed,including general data, classification of pathology, clinical stage and prognosis. MYCN gene amplification was detected by fluorescence in situ hybridization（FISH） and its relationship between pathological characteristics and prognostic significance was analyzed. Results There was 154 eases of NT aged 1 day to 11 years,with a mean age of 26.1 months, and the median age of 20.5 months. Male and female ratio was 1.48 ： 1.00. According to International Neuroblastoma Staging System （INSS） ,20 cases were of stage Ⅰ （ 13.0% ） ,23 cases of stage Ⅱ（ 14.9% ） ,43 cases of stageⅢ（ 27.9% ）, 64 cases of stage Ⅳ（41.6% ） and 4 cases of Ⅳs （2.6%）. There were 72 cases（46.8% ） with favorable histology,and 82 cases（53.2% ） with unfavorable histology. MYCN amplification was found in 20 cases （ 13.0% ） and the signal ratio of MYCN and chromosome 2 （CEP2） was 4.08 -43.29. One hundred and thirty - four cases of MYCN non - amplification included MYCN gain in 91 cases（68.0% ） ,MYCN negative in 43 cases（32.0% ）. MYCN expression showed the significant differences in ages, neuroblastoma type, international neuroblastoma pathology classification （INPC）, mitosis karyorrhexis index （MKI）, and clinical stages （all P 〈 0.05 ）. No significant difference was found in gender（ P 〉 0.05 ）. Of 20 MYCN amplification cases ,4 cases （20.0%） survived and 16 cases （80.0%） died ,and the overall survival rate was 20. 0% （4/20 cases） ,with survival time was （ 17.10 ±2.24） months;of 134 MYCN non - amplification cases,96 cases （71.6%） survived and 38 cases （28.4%） died,with survival time of （28.71 ±1.28 ）months. Survival analysis showed the cases with MYCN amplification had worse prognosis （Х^2 = 19. 596, P 〈 0.05 ）. Conclusions Patients with MYCN amplification
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2015年第23期1812-1815,共4页
Chinese Journal of Applied Clinical Pediatrics
基金
广东省医学科学技术研究基金（A2015484）
