相关实验视频
Updated: Jun 29, 2025

A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
[转移性染细胞瘤/副瘤患者的临床特征]
1Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People's Republic of China, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
转移性乳腺红细胞瘤/副细胞瘤 (PPGL) 往往涉及多系统传播和高基因突变率,特别是SDHB. 尽管有可能快速进展,但大多数转移PPGL的患者表现出良好的长期存活率.
科学领域:
- 内分泌学和瘤学的内分泌学和瘤学.
- 遗传学和分子生物学
- 临床医学 临床医学
背景情况:
- 乳色细胞瘤/副细胞瘤 (PPGL) 是一种罕见的神经内分泌瘤.
- 转移性PPGL带来了独特的临床挑战,需要进行详细的分析以改善管理.
- 了解基因格局和临床特征对于预测预后和指导治疗至关重要.
研究的目的:
- 综合分析被诊断患有转移性叶绿色细胞瘤/副腺细胞瘤 (PPGL) 的患者的临床特征.
- 确定转移性PPGL的关键预后因素和治疗模式.
- 调查转移PPGL的生殖线突变的流行率和影响.
主要方法:
- 从2018年1月至2023年8月期间接受治疗的250名转移性PPGL患者的临床数据的回顾性分析.
- 使用卡普兰-梅尔生存分析评估临床特征,治疗状态和预后.
- 检查生殖基因突变,评估生化标记物 (上腺素,上腺素,NSE) 和Ki-67指数.
主要成果:
- 26.4%的患者在初始诊断时发生了转移;20.1%的患者在手术后10年内发展了转移.
- 观察到高基因突变率 (55.2%),SDHB突变是最常见的 (76.5%的突变病例).
- 多系统转移 (85.6%) 是常见的,主要涉及骨 (60.8%);5年和10年生存率分别为88.0%和84.0%.
结论:
- 转移性PPGL经常与生殖基因突变有关,特别是SDHB.
- 瘤经常表现出多系统参与,起源于上腺之外,并具有高的Ki-67指数.
- 尽管具有侵略性特征,转移性PPGL通常显示出良好的预后,尽管部分患者的病情进展迅速.
更多相关视频
08:57Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
07:43Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
Published on: January 17, 2018
相关概念视频
Adrenal Gland Disorders
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...
Sympathetic Pathways: Collateral Ganglia and Adrenal Medulla
Collateral Ganglia
Sympathetic preganglionic axons reach the collateral ganglia along the route of splanchnic nerves. These nerves bypass the sympathetic trunk and communicate with sympathetic postganglionic neurons housed in the prevertebral ganglia. These ganglia supply the organs of the abdominopelvic cavity.
The greater splanchnic nerve, formed by the...
Sympathetic Signaling
Sympathetic preganglionic fibers release the neurotransmitter acetylcholine (ACh) onto the ganglionic neurons in the...
Peripheral Nervous System: Ganglia and Nerves
Nerves
The nerve is a bundle of axons that serves as the communication highway in the PNS. Each nerve is ensheathed in a protective layer of connective tissue called the epineurium. This outermost layer safeguards the nerve and supports the...
Drugs Acting on Autonomic Ganglia: Blockers
Disorders of the Autonomic Nervous System
Raynaud's disease, also known as Raynaud's...