Cushing’s syndrome (CS) refers to the clinical features of prolonged pathological glucocorticoid excess. About 10–20% of individuals with CS have ectopic CS (ECS), that is, an adrenocorticotropin (ACTH)-producing tumour outside the pituitary gland. ACTH-secreting neuroendocrine neoplasia (NENs) can arise from many organs, although bronchial NEN, small cell lung cancer (SCLC), pancreatic NEN, thymic NEN, medullary thyroid cancer (MTC), and pheochromocytoma are the most common. Patients with ECS frequently present with severe hypercortisolism. The risk of life-threatening complications is high in severe cases, unless the hypercortisolism is effectively treated. A good outcome in ECS requires a methodical approach, incorporating prompt diagnosis, tumour localization, control of cortisol excess, and resection of the primary tumour when possible.

中文翻译：

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异位库欣综合征的临床观点

库欣综合征（CS）是指长期病理性糖皮质激素过量的临床特征。大约 10-20% 的 CS 患者患有异位 CS (ECS)，即垂体外产生促肾上腺皮质激素 (ACTH) 的肿瘤。 ACTH 分泌型神经内分泌肿瘤 (NEN) 可由许多器官引起，但最常见的是支气管 NEN、小细胞肺癌 (SCLC)、胰腺 NEN、胸腺 NEN、甲状腺髓样癌 (MTC) 和嗜铬细胞瘤。 ECS 患者经常出现严重的皮质醇增多症。在严重的情况下，危及生命的并发症的风险很高，除非皮质醇增多症得到有效治疗。 ECS 的良好结果需要有条不紊的方法，包括及时诊断、肿瘤定位、控制皮质醇过量以及尽可能切除原发肿瘤。