恶性帕拉格林瘤与卡尔瓦里转移呈现与反复发生的甲基荷胺诱导心肌病变
Beatrice A Brumley1, Run Yu1, Shadfar Bahri2
1Division of Endocrinology, Diabetes, and Metabolism, University of California, Los Angeles (UCLA), David Geffen School of Medicine, Los Angeles, California.
AACE clinical case reports
|February 3, 2025
概括
恶性偏角质瘤 (PGL) 很少扩散到头骨. 这一案例显示了成功的手术切除形PGL转移,强调了早期检测和干预的积极监测.
科学领域:
- 神经瘤学神经瘤学
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
背景情况:
- 恶性偏角质瘤 (PGLs) 和染细胞瘤通常转移到内脏器官,骨和淋巴结,部的参与非常罕见.
- 早期检测和多学科管理对于患有PGL的患者至关重要,因为它们可能具有侵略性行为和关键的解剖位置.
研究的目的:
- 报告一种罕见的,来自恶性偏角质瘤的瘤转移病例.
- 讨论罕见的头骨PGL转移的管理挑战和治疗选择.
主要方法:
- 一名31岁的男性,有前史患有大心周PGL和转移性疾病,出现高血压危机和神经缺陷的症状.
- 诊断工作包括生物化学测试 (甲氨酸和甲氨酸水平升高) 和成像 (CT/MRI),揭示了大骨损伤和骨损伤.
- 进行了额头骨病变的手术切除,随后进行了辅助成像和多学科团队咨询.
主要成果:
- 病理学证实了在切除的骨病变中转移性偏角质瘤.
- 术后成像显示,手术部位没有复发,但发现了稳定的部病变和新的骨转移.
- 该患者是受体放射性核素治疗 (PRRT) 的候选人.
结论:
- 这一案例强调了对PGL患者的警监测的重要性,以促进早期检测转移性疾病,即使在像骨这样的罕见部位.
- 皮PGL转移的管理是复杂的,可能涉及手术,PRRT是潜在的治疗选择,特别是当放射治疗是禁忌时.
相关概念视频
Adrenal Gland Disorders
1.3K
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...
1.3K
Transducer Mechanism: Enzyme-Linked Receptors
2.3K
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:
2.3K
Tumor Progression
6.2K
Tumor progression is a phenomenon where the pre-formed tumor acquires successive mutations to become clinically more aggressive and malignant. In the 1950s, Foulds first described the stepwise progression of cancer cells through successive stages.
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...
6.2K
Mechanism of Cardiac Arrhythmias
882
Arrhythmias are irregular heart rhythms occurring when the heart's electrical impulses become abnormal. These disturbances can lead to various symptoms, depending on their severity and the underlying cause. Some common factors contributing to arrhythmias include hypoxia, ischemia, electrolyte imbalances, excessive catecholamine exposure, drug toxicity, and muscle overstretching. Arrhythmias can be classified into two main types based on the rate and site of origin of abnormal heart rhythms.
882
Heart Failure Drugs: Inotropic Agents
505
Positive inotropic agents are commonly used as the first line of treatment for heart failure. One such agent is digoxin, derived from the genus Digitalis, which has been known for centuries but effectively utilized since 1785. However, these cardiac glycosides can have potentially toxic effects due to their mechanism of action, which involves inhibiting Na+/K+-ATPase and increasing contractility. Digoxin is absorbed orally and distributed in various tissues, including the CNS. It has a long...
505


