儿红细胞瘤和儿童和青少年的偏角细胞瘤
Teresa Stachowicz-Stencel1, Natalia Pasikowska1, Anna Synakiewicz1
1Department of Pediatrics, Hematology and Oncology, Medical University of Gdansk, Gdańsk, Poland.
Acta biochimica Polonica
|September 17, 2023
概括
儿科发红细胞瘤和偏角细胞瘤 (PPGL) 是源自色氨酸细胞的罕见瘤. 诊断包括评估甲基胺,成像和基因检测,手术作为主要治疗方法.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 在瘤学瘤学.
- 医疗成像医学成像
背景情况:
- 乳红细胞瘤 (PPC) 和偏角细胞瘤 (PGL) 是源自染色细胞的罕见神经内分泌瘤,影响儿童群体 (PPGL).
- 这些瘤可以表现为过多的甲基荷兰胺 (高血压,头痛,出汗,心) 或质量效应的症状.
- 儿童的PPGL经常与家族综合征和特定的遗传突变有关 (例如,RET,VHL,NF1,SDHx).
研究的目的:
- 提供关于儿科发红细胞瘤和偏角细胞瘤的全面概述.
- 突出诊断挑战和成像和遗传评估方面的进展.
- 讨论当前和新兴的治疗策略以及长期随访的重要性.
主要方法:
- 对儿科PPGL的临床表现,诊断工作和治疗方式的审查.
- 重点是生物化学测试 (甲基醇胺和代谢物),放射性成像 (CT,MRI) 和核医学 (68Ga-DOTATATE).
- 对家族性综合征的基因测试和向治疗的作用的讨论.
主要成果:
- 生物化学和成像研究对于诊断至关重要,核成像显示高灵敏度.
- 基因检测对于识别家族性综合征和指导治疗至关重要.
- 手术切除仍然是主要的治疗方法,针对性治疗对复杂病例有希望.
结论:
- 准确诊断儿科PPGL需要一个多学科的方法,整合临床,生化,成像和遗传数据.
- 68Ga-DOTATATE PET/CT是用于全面评估的首选核成像追踪剂.
- 长期监测对于早期检测复发或转移性疾病至关重要.
更多相关视频
07:39Co-culture of Glutamatergic Neurons and Pediatric High-Grade Glioma Cells Into Microfluidic Devices to Assess Electrical Interactions
Published on: November 17, 2021
3.5K
07:43Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas
Published on: January 17, 2018
18.9K
相关概念视频
01:27Adrenal Gland Disorders
1.6K
Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
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...
1.6K
00:59The Parathyroid Glands
2.2K
The two pairs of parathyroid glands embedded within the posterior surface of the thyroid gland are restricted by a dense capsule around them. These glands comprise two distinct cell populations—parathyroid oxyphil and parathyroid principal cells- pivotal in calcium homeostasis.
Oxyphil cells, whose functions remain elusive, emerge during late puberty, adding a layer of complexity to the parathyroid gland's intricacies. In contrast, principal parathyroid cells undertake a vital role by...
Oxyphil cells, whose functions remain elusive, emerge during late puberty, adding a layer of complexity to the parathyroid gland's intricacies. In contrast, principal parathyroid cells undertake a vital role by...
2.2K
01:24Peripheral Nervous System: Ganglia and Nerves
2.1K
The Peripheral Nervous System (PNS) is a crucial component of the body's neural network, extending beyond the central nervous system (CNS) to bridge the gap between the CNS and the external environment. It encompasses nerves, ganglia, and sensory receptors.
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...
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...
2.1K
01:28Sympathetic Pathways: Collateral Ganglia and Adrenal Medulla
1.3K
The sympathetic pathways of the collateral ganglia and adrenal medulla serve unique but interconnected roles in the sympathetic response.
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...
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...
1.3K
01:27Transducer Mechanism: Enzyme-Linked Receptors
2.5K
Enzyme-linked receptors are cell-surface receptors acting as an enzyme or associating with an enzyme intracellularly. They make excellent drug targets. Drugs can bind to the extracellular ligand-binding domain or directly affect their enzymatic domain and alter their activity.
Major types that are helpful drug targets include:
Major types that are helpful drug targets include:
2.5K
01:15Hypoglycemia and Glucagon
294
Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
294