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婴儿期神经母细胞瘤临床治疗及预后分析

Therapeutic Strategies and Prognosis of Neuroblastoma in Infants

  • 摘要:
    目的  总结单中心年龄≤12月龄婴儿期神经母细胞瘤(NB)患者临床特征,分析影响预后的相关因素。
    方法  回顾性分析2014年1月—2022年12月于上海市儿童医院血液肿瘤科诊断治疗的婴儿NB(≤12月龄)临床资料,对其临床特征及影响预后因素进行统计分析,采用χ2检验比较组间率,对数秩检验进行单因素预后分析,Kaplan-Meier法评估生存情况。
    结果  共纳入42例患者,低危组仅行手术切除,中危组予手术联合化疗±维持治疗,高危组予手术联合化疗±维持治疗±放疗。5年无事件生存率(EFS)(92.7±4.9)%,5年总生存率(95.2±3.6)%,仅2例因肿瘤复发或进展死亡。单因素分析示,MYCN扩增、初诊乳酸脱氢酶(LDH)≥5倍正常值均与预后不良相关(5年EFS 33.3% vs. 97.4%、60.0% vs. 97.3%,P< 0.0001P= 0.0035)。
    结论  婴儿期NB整体预后良好,但MYCN基因扩增及初诊时LDH显著升高提示预后较差。

     

    Abstract:
    Objective  To summarize the clinical characteristics and analyze prognostic factors of neuroblastoma (NB) in infants (≤12 months) at a single center.
    Methods  A retrospective analysis was conducted on the clinical data of infant patients (≤12 months) diagnosed with NB and treated between January 2014 and December 2022. Clinical features were analyzed, and comparisons between two sample rates were performed using the χ2 test. Univariate prognostic analysis was conducted using the log-rank test, and survival outcomes were analyzed using the Kaplan-Meier method.
    Results  A total of 42 infants (≤12 months) with NB were enrolled. Low-risk patients underwent surgical resection alone; intermediate-risk patients received surgery combined with chemotherapy with or without maintenance therapy; high-risk patients were treated with surgery and chemotherapy with or without maintenance therapy or radiotherapy. The 5-year event-free survival (EFS) rate was (92.7±4.9)%, and the 5-year overall survival rate was (95.2±3.6)%. Only two patients died because of tumor recurrence or progression. Univariate analysis identified MYCN amplification and the initial lactate dehydrogenase (LDH) level ≥ five times the upper limit of the normal were significantly associated with poor prognosis (5-year EFS: 33.3% vs. 97.4% and 60.0% vs. 97.3%, P< 0.0001 and P= 0.0035).
    Conclusion  Infant NB has a favorable overall prognosis. MYCN amplification and markedly elevated initial LDH are associated with poor outcomes.

     

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