双边上腺神经母细胞瘤:一种罕见的儿科表现的特殊模式
Mohamed Fawzy1,2, Gehad Ahmed3,4, Yasser Youssef1,2
1Pediatric Oncology Department, Children's Cancer Hospital Egypt-57357, Cairo, Egypt.
Discover oncology
|April 12, 2024
概括
双边上神经母细胞瘤 (BSN) 是罕见的,但埃及患者的结果各不相同. 临床和生物学因素显著影响BSN的存活率,需要进一步研究预后指标.
科学领域:
- 儿科瘤学 儿科瘤学
- 癌症生物学 癌症生物学
- 临床研究 临床研究
背景情况:
- 双边上神经母细胞瘤 (BSN) 是一种不常见的诊断.
- 以前的文献表明BSN的预后是有利的.
- 这项研究评估了埃及BSN患者的临床和生物特征.
研究的目的:
- 评估埃及患者中BSN的临床和生物特征.
- 分析这些特征与患者结果 (总生存率和无事件生存率) 之间的关系.
主要方法:
- 对2007年至2017年间诊断的33名BSN患者的回顾性分析.
- 组织活检,成像和骨髓在呈现时的评估.
- 对临床,人口统计,生物变量和与OS和EFS相关的风险组进行分析.
主要成果:
- 在神经母细胞瘤病例中,BSN占2%,其中72.7%为婴儿.
- 大多数患者 (75.8%) 呈现远程转移.
- 在10名患者中发现了扩大MYCN;风险分层包括15名高风险和15名中等风险患者.
- 高风险和中等风险患者的三年生存率分别为40.5%和83.9%.
结论:
- 治疗BSN与单方面疾病管理相似.
- 考虑保守性手术,保护上腺组织.
- 生物变量和疾病程度是关键的预后因素.
- 需要对BSN生物分析和上腺大小差异显著性的进一步研究.
相关概念视频
Anatomy of the Adrenal Glands
The adrenal or supra-renal glands, situated above the kidneys and aligned with the twelfth rib, are paired pyramid-shaped structures crucial for the body's stress response. During stress, these glands secrete hormones vital for adaptive physiological reactions.
These glands possess a distinctive yellow tinge due to the stored cholesterol and fatty acids required for hormone synthesis. They are encased in a fibrous capsule and cushioned by fat.
The adrenal gland comprises two distinct regions...
These glands possess a distinctive yellow tinge due to the stored cholesterol and fatty acids required for hormone synthesis. They are encased in a fibrous capsule and cushioned by fat.
The adrenal gland comprises two distinct regions...
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Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...


