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Jorge Alberto Ramírez-García¹, Gabriela Garza-García¹, Roopa Mehta¹

¹Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán", Mexico City 14080, Mexico.

JCEM case reports

|March 2, 2026

概括

宫外库辛综合征是高皮质醇症的罕见原因,需要及时诊断. 这一案例突出了管理侵袭性,宫外ACTH产生瘤的挑战,强调了一种多学科的方法,以获得更好的结果.

科学领域:

内分泌学在内分泌学.
在瘤学瘤学.
核医学是一种核医学.

背景情况:

宫外库希综合征是一种罕见但严重的疾病,导致高皮质醇.
早期识别和治疗对于预防严重并发症和死亡至关重要.

研究的目的:

为了提出一种具有挑战性的异胎性库希综合征病例,由于中神经内分泌瘤.
为了说明在管理侵略性,宫外ACTH产生瘤的诊断和治疗的复杂性.

主要方法:

一名33岁的男性患有严重的库辛综合征,接受了生物化学测试和高级成像.
诊断工具包括24小时尿液自由皮质醇,ACTH水平和18F-TOC-PET/CT.
治疗涉及医疗管理 (基托可纳,体静止素类似物,埃托米达) 随后是除手术和双侧上腺切除术.

主要成果:

生物化学测试证实了高皮质醇和高ACTH和皮质醇的高皮质醇.
18F-TOC-PET/CT发现了一个大型的中间骨瘤.
最初的活检表明是一种低级的神经内分泌瘤,但后来的病理学揭示了更高级的转移性疾病.
由于瘤的位置,攻击性和转移性传播,管理是复杂的.

结论:

严重的高皮质醇症通常是由于子宫外的ACTH产生,并可能危及生命.
侵略性瘤和具有挑战性的位置需要一个多学科的战略,以有效管理.
这一案例强调了对子宫外库辛综合征进行全面评估和量身定制治疗的重要性.

关键词:

埃托米达的输液是为了输液.恶性瘤是一种恶性瘤.严重的高皮质醇主义.

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