胰腺ACTH过分分泌和下垂体腺瘤
Chiara M Bettale1, Jason W Allen2,3, Zaid K Mahdi4
1Emory University School of Medicine, Atlanta, GA 30322, USA.
JCEM case reports
|November 1, 2023
概括
一种罕见的胰腺神经内分泌瘤通过分泌ACTH引起库希综合征,导致高血压紧急情况和心肌梗塞. 手术去除症状,突出了需要进行彻底的内分泌检测.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 病理学 病理学 病理学
背景情况:
- 库希综合征的特征是过度暴露于皮质醇.
- 宫外ACTH分泌可以模仿垂体依赖的库辛病.
研究的目的:
- 报告一种极为罕见的异位ACTH分泌胰腺神经内分泌瘤与非功能性垂体腺瘤共存的病例.
- 在复杂的库辛综合征病例中强调动态内分泌检测的诊断效用.
主要方法:
- 一个55岁妇女的临床病例介绍,患有高血压急诊和心肌梗塞.
- 生物化学评估包括尿液和唾液皮质醇和ACTH水平.
- 动态内分泌检测 (皮质激素释放激素刺激试验).
- 下垂体和腹部成像 (MRI,CT).
- 胰腺质量的手术切除和组织病理学分析 (ACTH的免疫组织化学).
主要成果:
- 尿液和唾液中的皮质醇和ACTH水平升高.
- pituitary macroadenoma 被发现,但ACTH和皮质醇对皮质otropin释放激素没有反应.
- 大型胰腺质量被诊断为世卫组织第2级远部胰腺神经内分泌瘤. 染色ACTH.
- 术后缓解库辛症状,ACTH和皮质醇水平正常化,需要补充皮质.
- 在3.5年的随访期间,稳定的垂体腺瘤大小和其他垂体轴的正常功能.
结论:
- 来自胰腺神经内分泌瘤的异位ACTH分泌可以模仿垂体依赖的库辛综合征.
- 动态内分泌检测对于区分库辛综合征中ACTH的来源至关重要.
- 宫外ACTH源的手术治疗导致生物化学和临床缓解.
- 当使用适当的测试时,非功能性垂体腺瘤可以与异位ACTH分泌瘤共存,而不会影响诊断途径.
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