Chadli A, Alami L, El Aziz S, Lazghad A, El Ghomari H, Kafih M, Essodegui F, Jabri L, Farouqi A, Marouan F
Service d'Endocrinologie et Maladies Métaboliques, CHU Ibn Rochd, Casablanca, Maroc.
Ann Endocrinol (Paris). 2006 Jun;67(3):253-8. doi: 10.1016/s0003-4266(06)72595-1.
Cushing paraneoplasic syndrome is a rare cause of hypercorticism. We report a case of 35 year-old man presenting with Cushing's syndrome characterized by severe signs of hypercorticism and hypokalemia. Endocrine investigations were suggestive of an hypercortisolism linked to an ectopic adrenocorticotropic (ACTH) secretion, both at baseline (mean ACTH levels=275 pg/ml, urinary free cortisol excretion=3.898 mmol/24 h) and after pharmacodynamic testing (lack of inhibition of ACTH by dexamethasone). Thoracic tomodensitometric examination revealed a 15 mm tumor corresponding to a neuroendocrine pulmonary carcinoid with positive immunostaining for chromogranin A. Postoperative ACTH measurement was undetectable, plasma cortisol and free urinary cortisol were also decreased after tumor resection suggesting complete tumor removal. This case report illustrates the characteristics of paraneoplasic Cushing syndrome due to ACTH secreting pulmonary neuroendocrine carcinoid.
库欣副肿瘤综合征是皮质醇增多症的罕见病因。我们报告一例35岁男性,表现为库欣综合征,其特征为严重的皮质醇增多症和低钾血症体征。内分泌检查提示存在与异位促肾上腺皮质激素(ACTH)分泌相关的皮质醇增多症,无论是在基线时(平均ACTH水平=275 pg/ml,尿游离皮质醇排泄量=3.898 mmol/24小时)还是在药效学检测后(地塞米松对ACTH无抑制作用)。胸部断层扫描检查发现一个15毫米的肿瘤,为神经内分泌性肺类癌,嗜铬粒蛋白A免疫染色呈阳性。术后检测不到ACTH,肿瘤切除后血浆皮质醇和尿游离皮质醇也降低,提示肿瘤已完全切除。本病例报告阐述了由分泌ACTH的肺神经内分泌类癌引起的副肿瘤性库欣综合征的特征。