异位 ACTH 综合征 (EAS) 仍然是内分泌科医生面临的最严峻的诊断和治疗挑战之一。胸腺神经内分泌肿瘤占所有 EAS 病例的 5%-10%。我们报告了一名 31 岁女性的独特病例，她患有严重的 EAS，由原发性转移性联合大细胞神经内分泌癌和非典型胸腺类癌引起。该患者出现严重的高皮质醇血症，通过持续输注依托咪酯成功控制了病情。复杂的成像最初未能检测出胸腺病变；然而，它显示出一个巨大的、不均匀的、代谢活跃的左肾上腺肿块浸润隔膜，怀疑是原发性疾病的起源。患者接受了单侧肾上腺切除术，导致高皮质醇血症得到缓解。病理报告显示腺瘤伴肾上腺梗死和坏死。几周后的后续影像学研究最终发现了胸腺肿瘤。由于局部侵袭和快速进展，只能部分切除胸腺肿瘤，患者开始接受放疗和化疗。版权所有 © 2024 Dzialach、Wojciechowska-Luzniak、Maksymowicz 和 Witek。

Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%-10% of all EAS cases. We report a unique case of a 31-year-old woman with severe EAS caused by primary metastatic combined large-cell neuroendocrine carcinoma and atypical carcinoid of the thymus. The patient presented with severe hypercortisolemia, which was successfully controlled with continuous etomidate infusion. Complex imaging initially failed to detect thymic lesion; however, it revealed a large, inhomogeneous, metabolically active left adrenal mass infiltrating the diaphragm, suspected of primary disease origin. The patient underwent unilateral adrenalectomy, which resulted in hypercortisolemia resolve. The pathology report showed an adenoma with adrenal infarction and necrosis. The thymic tumor was eventually revealed a few weeks later on follow-up imaging studies. Due to local invasion and rapid progression, only partial resection of the thymic tumor was possible, and the patient was started on radio- and chemotherapy.Copyright © 2024 Dzialach, Wojciechowska-Luzniak, Maksymowicz and Witek.