PHOX2B蛋白、MYCN基因与外周神经母细胞性肿瘤临床病理特征及预后的关系

Relationship between PHOX2B protein and MCYN gene with clinicopathological features and prognosis of peripheral neuroblastic tumors

目的探讨同源异型盒2B(PHOX2B)蛋白和N-MYC致癌基因(MYCN基因)状态与外周神经母细胞性肿瘤(pNT)临床病理特征和预后的关系。方法采用免疫组织化学染色化法检测41例pNT患儿组织中PHOX2B蛋白的表达,荧光原位杂交技术检测MYCN基因表达状态,并使用连续校正χ2检验和Kaplan-Meier法分析两者与临床病理特征及预后的关系。结果免疫组织化学染色显示PHOX2B蛋白主要定位于细胞核内,在pNT组织中阳性表达率为82.9%,其表达水平与pNT发病年龄、分化程度及预后有关(P<0.05),PHOX2B蛋白表达强度与MYCN基因状态无关(P>0.05)。MYCN基因扩增患儿生存期短于未扩增患儿(P<0.05)。结论PHOX2B蛋白对pNT具有诊断意义,可以作为pNT可靠的诊断标志物;MYCN基因状态与患儿生存期有关,可以帮助判断pNT患儿预后情况。

Objective To investigate the relationship between the paired like homeobox 2B(PHOX2B)protein and N-MYC oncogene(MYCN gene)status with the clinicopathological features and prognosis of peripheral neuroblastic tumors(pNT).Methods The PHOX2B protein expression in pNT tissues of 41 children cases of pNT was detected by immunohistochemical staining,the MYCN gene expression status was detected by fluorescence in situ hybridization technique,and their relationship with clinicopathological features and prognosis was analyzed by continuous correction chi-square test and Kaplan-Meier method.Results The immunohistochemical staining showed that PHOX2B protein was mainly localized in the cell nucleus,and the positive expression rate in pNT tissue was 82.9%.Its expression level was related to the onset age,differentiation degree and prognosis of pNT(P<0.05),and the intensity of PHOX2B protein expression was not related to the MYCN gene status(P>0.05).The survival stage in the children patients with MYCN gene amplification was shorter than that in the children patients with no MYCN gene amplification(P<0.05).Conclusion PHOX2B protein has the diagnostic significance for pNT and could be used as a reliable diagnostic marker for pNT;MYCN gene status is related to the survival period in the children patients,which can help to judge the prognosis of pNT children patients.