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资源有限环境下神经母细胞瘤的预后因素

Prognostic factors of neuroblastoma in limited-resource settings.

作者信息

Pinto Roberta Gomes Ribeiro Gonçalves, Lins Mecneide Mendes, Pereira Kaline Maria Maciel de Oliveira, Meira Ticiana Ester Mattos Pascoal, Coêlho Eduarda Coutinho Albuquerque Neiva, Moraes Alice Rodrigues Barbosa de, Batista Marina Lundgren de Melo, Pereira Leticia Ribeiro Maciel, Mello Maria Júlia Gonçalves de

机构信息

Instituto de Medicina Integral Prof. Fernando Figueira - Recife (PE), Brazil.

出版信息

Rev Paul Pediatr. 2025 Jul 28;43:e2024200. doi: 10.1590/1984-0462/2025/43/2024200. eCollection 2025.

DOI:10.1590/1984-0462/2025/43/2024200
PMID:40736099
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12313317/
Abstract

OBJECTIVE

This study aimed to analyze survival and risk factors for death in children with neuroblastoma that may contribute to a viable risk classification for low-income countries.

METHODS

A historic cohort involving patients under 19 years of age with neuroblastoma was followed at a reference center in Northeast Brazil between July 2005 and July 2020. Data on sociodemographic, were collected. The outcomes studied were recurrence, disease progression, and five-year mortality. Multivariate analysis of Cox proportional hazards for death was performed. Overall and eventfree survivals were evaluated using the Kaplan-Meier method and the comparison between the groups studied, by the log-rank test.

RESULTS

Patients (n=126) were predominantly female with a median age of 26.5 months. Most presented primary adrenal, tumor stage 4 according to the International Neuroblastoma Staging System, unfavorable histology, and median serum lactate dehydrogenase (LDH) levels of 640.5 U/L. LDH≥640.5 U/L (hazard ratio [HRa] 2.49; 95% confidence interval [CI] 1.57-3.95; p<0.001) and stage 4 (HRa 1.67; 95%CI 1.00-2.78; p=0.047) were identified as risk factors for death. The overall survival was 32.4%, showing distinct curves regarding LDH serum levels and staging (log-rank p<0.05); the event-free survival was 26.3%.

CONCLUSIONS

Elevated LDH serum levels were a risk factor for five-year mortality, and can be utilized as a prognostic marker in resource-limited settings.

摘要

目的

本研究旨在分析神经母细胞瘤患儿的生存情况及死亡风险因素,这些因素可能有助于为低收入国家制定可行的风险分类标准。

方法

2005年7月至2020年7月期间,在巴西东北部的一个参考中心对一个涉及19岁以下神经母细胞瘤患者的历史性队列进行了随访。收集了社会人口统计学数据。研究的结局指标为复发、疾病进展和五年死亡率。对死亡进行了Cox比例风险多因素分析。采用Kaplan-Meier法评估总生存率和无事件生存率,并通过对数秩检验比较研究组之间的差异。

结果

患者(n = 126)以女性为主,中位年龄为26.5个月。大多数患者表现为原发性肾上腺肿瘤,根据国际神经母细胞瘤分期系统为4期,组织学类型不良,血清乳酸脱氢酶(LDH)中位水平为640.5 U/L。LDH≥640.5 U/L(风险比[HRa] 2.49;95%置信区间[CI] 1.57 - 3.95;p < 0.001)和4期(HRa 1.67;95%CI 1.00 - 2.78;p = 0.047)被确定为死亡风险因素。总生存率为32.4%,显示出LDH血清水平和分期的不同曲线(对数秩p < 0.05);无事件生存率为26.3%。

结论

血清LDH水平升高是五年死亡率的一个风险因素,可在资源有限的环境中用作预后标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e91/12313317/27b6265d4814/1984-0462-rpp-43-e2024200-gf01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e91/12313317/27b6265d4814/1984-0462-rpp-43-e2024200-gf01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e91/12313317/27b6265d4814/1984-0462-rpp-43-e2024200-gf01.jpg

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MYCN protein stability is a better prognostic indicator in neuroblastoma.MYCN 蛋白稳定性是神经母细胞瘤预后的一个更好的指标。
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