儿科多发性肺母细胞瘤死亡率的预测因素:监测流行病学和最终结果数据库分析

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概括

腹肺母细胞瘤 (PPB) 是一种罕见的儿科癌症. 晚期,需要化疗和女性性别是PPB患者死亡率的关键预测因素.

科学领域:

儿科瘤学儿科瘤学
罕见的癌症罕见的癌症
瘤生物学瘤生物学

背景情况:

腹肺母细胞瘤 (PPB) 是一种罕见的儿科恶性瘤,与DICER1基因突变有关.
早期的PPB (I型) 模仿婴儿的良性肺部疾病,而后期的PPB会影响较大的儿童.
准确的诊断和对预后因素的理解对于有效的管理至关重要.

研究的目的:

确定诊断为多发肺母细胞瘤 (PPB) 的儿科患者死亡率的预测因素.
分析2000年至2019年期间美国0-19岁PPB患者的死亡趋势.

主要方法:

对监测流行病学和最终结果 (SEER) 数据库 (2000-2019) 的回顾性分析.
包括诊断为PPB的儿科患者 (0-19岁) (ICD-O-3代码8973/3).
使用后勤回归的死亡风险预测方程的开发.

主要成果:

在71例PPB病例中,有16例 (22%) 导致死亡.
死亡率与晚期 (P=0.029),化疗需求 (P=0.047) 和女性性别 (P=0.019) 有显著的相关性.
结合阶段,化疗和性别的预测方程获得了82%的准确性.

结论:

PPB需要仔细区分和良性肺形,以防止进展.
先进的PPB阶段需要密集治疗 (化疗,放射) 并与较差的结果相关.
开发的预测模型有助于评估儿科PPB患者的死亡风险.

关键词:

DICER 1 1 的意思是说.pleuropulmonary 囊原体瘤是一个多重肺细胞瘤.预后预后预后

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