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一例由十二指肠产生异位促肾上腺皮质激素的类癌继发的库欣综合征。

A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum.

作者信息

Uecker John M, Janzow Matthew T

机构信息

Naval Medical Center Portsmouth, Portsmouth, Virginia, USA.

出版信息

Am Surg. 2005 May;71(5):445-6.

Abstract

Cushing syndrome caused by adrenocorticotropic hormone (ACTH) production from solid tumors can result in life-threatening hypercortisolemia. Ectopic ACTH production is most commonly associated with bronchial carcinoids and squamous cell carcinoma of the lung. We report a case of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum. The patient presented to an outside hospital in hypertensive crisis and diabetic ketoacidosis. After stabilization, diagnostic studies including a serum cortisol level, and computed tomography (CT) scans of the head, chest, abdomen, and pelvis revealed hypercortisolemia and a large mass in the head of the pancreas. Pancreaticoduodenectomy was performed. Pathologic investigation revealed a 1-cm carcinoid of the duodenum with two large metastatic lymph nodes near the head of the pancreas. This is the first reported case in the English literature of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum.

摘要

由实体瘤产生促肾上腺皮质激素(ACTH)引起的库欣综合征可导致危及生命的高皮质醇血症。异位ACTH分泌最常与支气管类癌和肺鳞状细胞癌相关。我们报告一例由十二指肠类癌异位分泌ACTH引起的库欣综合征病例。该患者因高血压危象和糖尿病酮症酸中毒就诊于外院。病情稳定后,包括血清皮质醇水平检测以及头部、胸部、腹部和骨盆的计算机断层扫描(CT)等诊断性检查显示存在高皮质醇血症以及胰腺头部有一个大肿块。遂行胰十二指肠切除术。病理检查发现十二指肠有一个1厘米的类癌,在胰腺头部附近有两个大的转移性淋巴结。这是英文文献中首例报道的由十二指肠类癌异位分泌ACTH引起的库欣综合征病例。

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