从食道神经内分泌癌引起的异位ACTH产生,导致库辛综合征
Yifei Zhang1, Xuchen Zhang2, Sachin Majumdar3
1Cancer, Smilow Cancer Hospital at Yale New Haven, New Haven, CT, USA yifei.k.zhang@yale.edu.
BMJ case reports
|February 4, 2026
概括
异位库希综合征 (ECS) 很少可能是由食道神经内分泌癌 (NEC) 引起的. 这一案例凸显了诊断方面的挑战,以及对这种侵袭性疾病的迅速,多学科治疗的需要.
科学领域:
- 内分泌学 在内分泌学.
- 在瘤学瘤学.
- 胃肠病学 胃肠病学
背景情况:
- 异位库希综合征 (ECS) 是由于非垂体瘤分泌过多的上腺皮质激素 (ACTH),导致高皮质醇.
- ECS通常与小细胞肺癌和胰腺神经内分泌瘤有关.
- oesophageal神经内分泌癌 (NEC) 是一种非常罕见的ECS病因,占神经内分泌瘤的不到0.04%.
研究的目的:
- 报告一种罕见的ECS病例,该病例是食道局部发达大细胞NEC的次要病例.
- 为了强调与ECS相关的诊断困难在不常见的瘤类型.
- 突出早期检测和综合管理对于改善患者治疗结果的重要性.
主要方法:
- 一个60多岁的男性患者的病例报告.
- 对于食道局部发达的大细胞NEC的初始治疗.
- 监测ECS和高皮质醇症的发展.
主要成果:
- 尽管患者最初接受了食道NEC的治疗,但患者的ECS仍在发展.
- 食道NEC的大细胞亚型特别罕见,并且与预后不佳有关.
- 患者的病情迅速恶化,尽管内分泌导向治疗,由于疾病的积极进展.
结论:
- 输卵管NEC是一种罕见但潜在的异位库希综合征的原因.
- 早期识别和多学科管理对于优化罕见ECS病例患者的治疗结果至关重要.
- 这一案例强调了与罕见瘤相关的ECS的诊断挑战和攻击性.
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