异位 adrenocorticotropic 激素 (ACTH) -依赖于嗅觉神经母细胞瘤的二次库希综合征:一个罕见的实体
1Division of Endocrinology, Diabetes, and Metabolism, Department of Internal Medicine, Community Health Partners, Fresno, CA.
AACE endocrinology and diabetes
|December 30, 2025
概括
由嗅觉神经母细胞瘤 (ONB) 引起的异位 adrenocorticotropic 激素 (ACTH) 依赖的库希综合征是罕见的. 早期诊断和多学科治疗,包括手术和放射治疗,导致成功的缓解和改善的结果.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 耳鼻喉科 耳鼻喉科 耳鼻喉科
背景情况:
- 异位 adrenocorticotropic 激素 (ACTH) 依赖的库希综合征是由非垂体瘤过度产生皮质醇引起的.
- 嗅觉神经母细胞瘤 (ONB) 是一种罕见的鼻恶性瘤,是异胎ACTH产生的一个不常见的原因,全球报告的病例少于25.
研究的目的:
- 为了呈现一个ACTH依赖的库希综合征的情况,这是ONB的次要原因.
- 强调诊断挑战,多学科管理和与这种罕见疾病相关的积极结果.
主要方法:
- 一名70岁的男性患有高皮质醇症和表症的症状,接受了全面的评估.
- 诊断工作包括生物化学测试,成像,下层状鼻采样和内镜瘤活检.
- 治疗包括手术前皮质醇抑制,内镜瘤切除和辅助放射治疗.
主要成果:
- 该患者呈现出经典的库辛综合征特征,高皮质醇症和升高的ACTH.
- 图像检测显示出一种状鼻腔质量,被证实为海姆斯2级ONB.
- 在治疗后,患者实现了生化缓解,Cushingoid症状的消失和HPA轴的恢复.
结论:
- 一种系统的诊断方法对于识别罕见的宫外ACTH来源,如ONB,至关重要.
- 为了有效管理这些复杂的案件,多学科的合作是必不可少的.
- 及时诊断,手术切除和辅助疗法可以导致内分泌缓解,并在患有ACTH分泌ONB.患者的患者中带来有利的长期结果.
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