Wang W Q, Ye L, Bi Y F, Zhao H Y, Sun S Y, Tang Z Y, Zhao Y J, Fang W Q, Chen Z Y, Chen K M, Jin X L, Ning G
Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, 197 Ruijin Er Lu, Shanghai, 20025 PR China.
J Endocrinol Invest. 2006 Apr;29(4):293-7. doi: 10.1007/BF03344098.
The ectopic ACTH syndrome (EAS) caused by thymic carcinoid is rare and its diagnosis remains a challenge to the endocrinologist. Here are six cases of EAS with a typical Cushing habitus accompanied by hyperpigmentation and hypokalemia. For all six patients, the high dose (8 mg) dexamethasone suppression test (HDDST) showed lack of suppression, computed tomography (CT) scanning documented anterior mediastinal masses, and the mediastenal tumors removed were confirmed as ACTH secreting thymic carcinoids by positive ACTH and NSE staining. Our data indicate that HDDST chest radiologic imaging and other laboratory examinations will greatly assist in diagnosing the thymic carcinoid-induced EAS at an earlier stage, which will significantly improve the long-term survival of the patient.
由胸腺类癌引起的异位促肾上腺皮质激素综合征(EAS)较为罕见,其诊断对内分泌科医生来说仍是一项挑战。本文报告6例伴有典型库欣体型、色素沉着和低钾血症的EAS病例。所有6例患者的高剂量(8mg)地塞米松抑制试验(HDDST)均显示无抑制,计算机断层扫描(CT)显示前纵隔肿块,切除的纵隔肿瘤经促肾上腺皮质激素(ACTH)和神经元特异性烯醇化酶(NSE)染色阳性,确诊为分泌ACTH的胸腺类癌。我们的数据表明,HDDST、胸部放射影像学检查和其他实验室检查将极大地有助于早期诊断胸腺类癌所致的EAS,这将显著提高患者的长期生存率。