儿科上腺皮质癌:临床特征和新辅助化疗的应用
Yu Lin1, Shen Yang1, Wei Yang1
1Department of Oncology Surgery, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, Beijing, 100045, China.
European journal of medical research
|October 9, 2023
概括
新辅助化疗在特定情况下对儿科上腺皮质癌 (ACC) 有希望. 这种方法与手术相结合,改善了ACC儿童的存活率,突出了全面内分泌评估的必要性.
科学领域:
- 儿科瘤学 儿科瘤学
- 内分泌学 在内分泌学.
- 手术瘤学手术瘤学
背景情况:
- 皮上腺癌 (ACC) 是儿童的一种罕见而侵袭性的恶性瘤.
- 早期诊断和有效的治疗策略对于改善儿科ACC的结果至关重要.
- 儿科ACC的临床特征和治疗疗效需要进一步研究.
研究的目的:
- 总结儿科上腺皮质癌 (ACC) 的临床特征.
- 探索新辅助化疗在患有ACC的儿童中的指示和有效性.
- 评估新辅助化疗对瘤反应和生存率的影响.
主要方法:
- 对49名患有上腺皮层瘤 (ACT) 的儿童进行了回顾性分析,其中40名被诊断患有ACC.
- 使用韦斯分级系统进行病理学评估.
- 使用RECIST 1.1标准和3DCT重建评估新辅助化疗反应.
主要成果:
- 异常的激素水平和性早成是儿科ACC的常见临床表现.
- 在7名患有大瘤或转移的儿童中,新辅助化疗导致了部分反应或稳定的疾病.
- 在所有接受3D体积重建的5名儿童中都观察到显著的瘤体积减少.
- 五年总生存率为90.0%,无事件生存率为81.5%.
结论:
- 综合性内分泌评估对于早期诊断儿科ACC至关重要.
- 手术和化疗是治疗儿科ACC的关键组成部分.
- 新辅助化疗对于符合特定标准的ACC儿童来说是一个可行的选择,包括大型瘤或远程转移.
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