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一个罕见的周期性库希综合征神秘说明了诊断原则

Yetunde Bernice Omotosho¹, Raven McGlotten¹, Karel Pacak²

¹Diabetes, Endocrinology and Obesity Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD 20892, USA.

JCEM case reports

|June 25, 2024

概括

这一案例突出了一个罕见的周期性库希综合征的例子,由ACTH分泌的染细胞瘤引起. 早期诊断和手术切除上腺瘤解决了这种情况.

科学领域:

内分泌学在内分泌学.
在瘤学瘤学.
内部医学内部医学

背景情况:

叶绿细胞瘤通常分泌甲基荷胺,但很少能产生ACTH,导致库辛综合征 (CS).
循环性CS呈现为间歇性高皮质醇症,使诊断变得困难.
乳房外的ACTH分泌物从染细胞瘤是一个不常见的原因的CS.

研究的目的:

报告一个周期性宫外ACTH分泌的病例从 pheochromocytoma.
强调诊断挑战和考虑在ACTH依赖的高皮质醇症中体红细胞瘤的重要性.

主要方法:

诊断工作包括荷尔蒙测定 (皮质醇,ACTH,甲氨酸),成像 (MRI,PET/CT),双边下层鼻抽样和甲抑制测试.
手术管理涉及右上腺切除术后的医疗准备与多克萨佐辛.
组织病理学和免疫组织化学证实了诊断.

主要成果:

患者呈现高血压,状特征,以及皮质醇和ACTH间歇性升高.
诊断测试表明宫外ACTH分泌,与高的诺米甲氨酸.
上腺切除术后,血压和荷尔蒙水平正常化,需要补充皮激素.

结论:

在依赖ACTH的高皮质醇症的情况下,特别是甲基内弗林水平升高和上腺质量升高的情况下,应考虑结核细胞瘤.
及时诊断和治疗ACTH分泌染细胞瘤可以改善患者的治疗结果.
这个案例说明了一个罕见但可治疗的周期性库希综合征的原因.

关键词:

周期性的库希综合征库希综合征宫外库希综合征宫外库希综合征叶染色细胞瘤 ( pheochromocytoma) 是一种致血性细胞瘤.

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