医学
库欣综合征
病态的
肺
嗜铬细胞瘤
甲状腺髓样癌
糖皮质激素
神经内分泌肿瘤
髓腔
甲状腺
内科学
病理
内分泌学
甲状腺癌
作者
Óskar Ragnarsson,C. Christofer Juhlin,David J. Torpy,Henrik Falhammar
标识
DOI:10.1016/j.tem.2023.12.003
摘要
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.
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