Lin Chin-Sheng, Yao Nai-Shun, Cheng Ming-Fang, Lin Shih-Hua
Division of Nephrology, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China.
Am J Med Sci. 2007 Dec;334(6):487-9. doi: 10.1097/MAJ.0b013e318068b25d.
Although ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a well-known paraneoplastic phenomenon, an association with large-cell neuroendocrine carcinoma of the lung (LCNEC) has not been reported. We describe a 63-year-old man with metastatic LCNEC to the left temporomandibular joint (TMJ) who presented with progressive muscle weakness and bilateral lower leg edema for 2 weeks. He did not have a typical Cushingoid appearance nor used diuretics. His newly noted hypertension, hypokalemia (plasma potassium (K) concentration 1.8 mEq/L) with renal K wasting, and metabolic alkalosis suggested a state of mineralocorticoid excess. His plasma renin activity and aldosterone concentrations were low, but cortisol and ACTH levels were extremely elevated, consistent with ACTH-dependent Cushing's syndrome. Nonsuppressible plasma cortisol level and normal sella turcica on magnetic resonance imaging pointed to EAS. A strongly positive stain for ACTH from the metastatic left TMJ mass supported LCNEC-related EAS. His hypokalemia and hypertension were controlled with spironolactone and K supplementation. This is the first reported case of EAS in LCNEC and should be kept in mind as a cause of hypokalemia in lung cancer patients.
尽管异位促肾上腺皮质激素（ACTH）综合征（EAS）是一种众所周知的副肿瘤现象，但尚未见其与肺大细胞神经内分泌癌（LCNEC）相关的报道。我们描述了一名63岁男性，其左颞下颌关节（TMJ）发生转移性LCNEC，出现进行性肌无力和双侧小腿水肿2周。他没有典型的库欣样外观，也未使用利尿剂。他新出现的高血压、伴有肾钾耗竭的低钾血症（血浆钾（K）浓度1.8 mEq/L）以及代谢性碱中毒提示存在盐皮质激素过多状态。他的血浆肾素活性和醛固酮浓度较低，但皮质醇和ACTH水平极度升高，符合ACTH依赖性库欣综合征。血浆皮质醇水平不可抑制且磁共振成像显示蝶鞍正常，提示为EAS。来自转移性左TMJ肿块的ACTH染色呈强阳性，支持与LCNEC相关的EAS。他的低钾血症和高血压通过螺内酯和补钾得到控制。这是首例报道的LCNEC相关EAS病例，应将其作为肺癌患者低钾血症的一个病因加以牢记。
