Niu Huilin, Xu Tao, Wang Fenghua, Chen Zhengrong, Gao Qiu, Yi Peng, Xia Jianqing
Department of Pathology, Guangzhou Women and Children's Medical Center, Guangzhou 510623, China. E-mail:
Zhonghua Bing Li Xue Za Zhi. 2015 Feb;44(2):111-7.
To summarize the clinicopathologic features of neuroblastic tumors (NT), and to explore the prognostic significance of MYCN amplification in NT.
The clinicopathologic data of 267 NT were reviewed. MYCN gene amplification was detected by fluorescence in situ hybridization (FISH) in 119 cases and the relationship with pathological characteristics and prognostic significance were analyzed.
The study included 267 cases of children NT from patients aged from 1 day to 13 years (median 27 months). The male to female ratio was 1.43. There were 38 cases (14.2%), 43 cases (16.1%), 71 cases (26.6%), and 115 cases (43.1%) of INSS stages I, II, III and IV respectively.Favorable histology group had 157 cases (59.9%); unfavorable histology group had 110 cases (40.1%).Of the 119 NT cases with MYCN FISH performed, 18 cases (15.1%) showed amplification and the signal ratio of MYCN to CEP2 was 4.08-43.29. One hundred and one cases of non-amplified MYCN included MYCN gain in 79 cases (66.3%) and MYCN negative in 22 cases (18.5%). MYCN expression showed significant difference (P = 0.000) between ages, gender, NT type and MKI, but not INPC and clinical stage (P > 0.05).Of the 18 cases with MYCN amplification, 3 were undifferentiated, and 15 poorly differentiated; 17 had high MKI and one moderate MKI. All 18 cases were in unfavorable histology group; the overall survival rate was 3/18, with an average survival time of (17.9 ± 2.4) months.Of the 101 MYCN non-amplification cases, the overall survival rate was 68.3% (69/101), with an average survival time of (29.8 ± 1.3) months. Survival analysis showed the cases with MYCN amplification had worse prognosis (P < 0.05).
NT were commonly diagnosed in early ages and easily to metastasize. Most of cases with favorable histology. The cases of MYCN amplification showed unfavorable histology, and the majority cases with high MKI; The patients with MYCN gene amplification had poor prognosis.
总结神经母细胞瘤（NT）的临床病理特征，探讨MYCN扩增在NT中的预后意义。
回顾267例NT的临床病理资料。采用荧光原位杂交（FISH）检测119例MYCN基因扩增情况，并分析其与病理特征及预后意义的关系。
本研究纳入267例年龄从1天至13岁（中位年龄27个月）的儿童NT。男女比例为1.43。国际神经母细胞瘤分期系统（INSS）Ⅰ、Ⅱ、Ⅲ和Ⅳ期分别有38例（14.2%）、43例（16.1%）、71例（26.6%）和115例（43.1%）。组织学预后良好组有157例（59.9%）；组织学预后不良组有110例（40.1%）。在119例行MYCN FISH检测的NT病例中，18例（15.1%）显示扩增，MYCN与着丝粒蛋白2（CEP2）的信号比为4.08 - 43.29。101例MYCN未扩增病例中，79例（66.3%）为MYCN增加，22例（18.5%）为MYCN阴性。MYCN表达在年龄、性别、NT类型和有丝分裂指数（MKI）之间存在显著差异（P = 0.000），但在国际神经母细胞瘤病理分类（INPC）和临床分期方面无显著差异（P > 0.05）。在18例MYCN扩增病例中，3例为未分化型，15例为低分化型；17例MKI高，1例MKI中等。18例均在组织学预后不良组；总生存率为3/18，平均生存时间为（17.9 ± 2.4）个月。在101例MYCN未扩增病例中，总生存率为68.3%（69/101），平均生存时间为（29.8 ± 1.3）个月。生存分析显示，MYCN扩增病例预后较差（P < 0.05）。
NT多在早期诊断，易发生转移。多数病例组织学预后良好。MYCN扩增病例组织学预后不良，多数病例MKI高；MYCN基因扩增患者预后差。
