儿童纵隔神经母细胞瘤临床特征分析

  目的  探讨纵隔神经母细胞瘤临床特征。  方法  收集2008年3月至2012年9月中国医科大学附属第四医院儿科收治的神经母细胞瘤110例, 其中26例肿瘤位于纵隔, 其他部位84例。将纵隔神经母细胞瘤的临床表现、肿瘤标志物、生物学预后因素与其他部位对比。  结果  纵隔组平均年龄25.5个月, 与其他部位组相近。初诊时纵膈组88.5%有症状, 高于其他部位组60.7%（P < 0.05）; 早期病例（Ⅰ、Ⅱ期）34.6%, 高于其他部位组8.3%（P < 0.05）; 血清NSE>100 ng/L者达21.4%, 低于其他部位组86.1%（P < 0.001）。纵隔组N-myc基因拷贝数均 < 10, 其他部位组N-myc基因拷贝数>10者为23.1%（P < 0.05）。纵隔组4年总生存率为80.0%, 其中局限性肿瘤（Ⅰ、Ⅱ、Ⅲ期）达100%, 分别高于其他部位组44.0%、82.0%。  结论  多数纵隔神经母细胞瘤表现出临床分期早（Ⅰ、Ⅱ期）和良好的生物学预后因素, 这些特征可能与纵隔神经母细胞瘤良好的预后相关。 

Characteristics of mediastinal neuroblastoma in children

  Objective  The present study aimed to study the clinical characteristics of mediastinal neuroblastoma.  Methods  From March 2008 to September 2012, the Fourth Affiliated Hospital of China Medical University admitted 110 cases of neuroblastoma, including 26 cases of mediastinal neuroblastoma and 84 cases of other neuroblastomas. The clinical manifestation, tumor markers, and biological prognostic factors of mediastinal neuroblastoma were compared with those of other neuroblastomas.  Results  The average age of patients in the mediastinal neuroblastoma group was 25.5 months, which was very similar to other neuroblastomas. About 88.5% of mediastinal neuroblastomas had newly diagnosed syndrome, and the corresponding value was 60.7% in other neuroblastomas (P < 0.05). The early-stage (Ⅰ and Ⅱ) cases in the mediastinal neuroblastoma group were 34.6%, higher than that in the other neuroblastomas, i.e., 8.3% (P < 0.05). About 21.4% of the serum NSE levels of the mediastinal neuroblastoma group increased by >100 ng/ L, which was lower than the 86.1% (P < 0.05) in other neuroblastomas. All cases of mediastinal neuroblastoma had an N-myc copy number < 10, whereas 23.1% of the other neuroblastomas had >10 copies (P < 0.05). The 4-year overall survival rate was 80.0% in the mediastinal group and 44.0% in the other neuroblastomas. Among the cases involving primary tumors in localized neuroblastomas, the 4-year survival rate was 100%, which was significantly higher than the 82.0% in other neuroblastomas.  Conclusion  The majority of mediastinal neuroblastoma cases found in the early clinical stage had favorable biological prognostic factors that may be associated with the prognosis of mediastinal neuroblastoma.