儿童神经母细胞源性肿瘤的形态及分子遗传学改变及临床意义

发布时间：2018-07-04 22:33

本文选题：神经母细胞瘤 + 节神经母细胞瘤　；参考：《临床与实验病理学杂志》2017年07期

【摘要】：目的探讨儿童神经母细胞源性肿瘤的组织学特征、免疫表型及MYCN扩增特点,判断其生物学行为及预后。方法分析100例神经母细胞瘤(neuroblastoma,NB)及节神经母细胞瘤(ganglioneuroblastoma,GNB)的组织学形态、免疫表型及60例MYCN扩增。结果 100例NB及GNB患儿,平均年龄2.7岁,男性多于女性;NB分为未分化型、差分化型、分化型;GNB分为混合型(iGNB)和结节型(nGNB)。免疫表型:神经母细胞不同程度的表达NSE、NF、PGP9.5、Syn、CgA;Schwannian细胞表达S-100、GFAP。11.67%的患者伴MYCN扩增,iGNB无MYCN扩增。结论神经母细胞源性肿瘤的诊断主要根据组织学形态,特殊检查(免疫组化、电镜和细胞遗传学)可以帮助鉴别未分化的母细胞成分。神经母细胞源性肿瘤的预后根据患者年龄、肿瘤的分型分期、分子遗传学改变等指标综合评估。
[Abstract]:Objective to investigate the histological features, immunophenotype and MYCN amplification characteristics of neuroblast-derived tumors in children, and to judge their biological behavior and prognosis. Methods the histological morphology, immunophenotype and MYCN amplification of 100 cases of neuroblastomaNB and ganglioneuroblastomaGNB were analyzed. Results 100 cases of NB and GNB with an average age of 2.7 years were classified as undifferentiated type and poorly differentiated type. Differentiated type GNB was divided into mixed type (iGNB) and nodular type (nGNB). Immunophenotype: the expression of NSENF-NFPGP9.5PGP9.5in the neuroblastoma cells and the expression of S-100mGFAP.11.67% of the patients with MYCN amplification and iGNB without MYCN amplification. Conclusion the diagnosis of neuroblast-derived tumors is mainly based on histological morphology, and special examination (immunohistochemistry, electron microscopy and cytogenetics) can help to differentiate undifferentiated mother cell components. The prognosis of neuroblast-derived tumors was evaluated according to age, tumor classification and stage, molecular genetic changes and so on.
【作者单位】：上海交通大学医学院附属新华医院病理科;
【分类号】：R739.4

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