Internal Medicine Department, Brugmann University Hospital, Brussels, Belgium.
Department of Pneumology, IRIS Hospitals South, Brussels, Belgium.
Acta Clin Belg. 2021 Oct;76(5):373-378. doi: 10.1080/17843286.2020.1734162. Epub 2020 Feb 23.
: Ectopic Cushing's syndrome is a very rare condition caused by an ACTH-secreting tumor outside the pituitary or adrenal glands, and the majority of these cases are encountered in the context of paraneoplastic syndromes. The ectopic source of ACTH secretion is not always obvious to detection and can be challenging. We report a rare case, in which a hidden ACTH-secreting carcinoid tumor of the lung caused a severe refractory hypokalemia, leading us to a race against time to locate the tumor.: A 33-year-old young male was admitted to the ICU for the management of a severe hypokalemia, and complains from several months of depression, increased weight, disabling non-radiating dorsal lower back pain and refractory arterial hypertension. The physical examination immediately suggested a Cushing's syndrome. The 24-h cortisoluria confirmed hypercortisolism and the increased ACTH level was oriented towards ACTH-dependent Cushing's syndrome. Thereafter, a dexamethasone suppression test was negative, indicating in favor of ectopic ACTH secretion. The etiological assessment via imaging and isotopes revealed a solitary pulmonary nodule at the right lower lobe estimated at 18 mm, the resection and anatomopathological analysis of which led to the diagnosis of carcinoid pulmonary tumor, and resolved hypercortisolism and its complications.: A delayed diagnosis of Cushing's syndrome result in a consequent morbi-mortality, mainly due to cardiovascular events. The optimal treatment for ectopic Cushing's syndrome is surgical resection, thus making the localization of the tumor a key element.
异位库欣综合征是一种非常罕见的疾病,由垂体或肾上腺以外的 ACTH 分泌肿瘤引起,大多数情况下见于副肿瘤综合征。异位 ACTH 分泌的来源并不总是易于检测,这可能具有挑战性。我们报告了一例罕见病例,其中肺隐匿性 ACTH 分泌类癌肿瘤导致严重难治性低钾血症,使我们争分夺秒地寻找肿瘤。
一名 33 岁的年轻男性因严重低钾血症被收入 ICU 进行治疗,并伴有数月的抑郁、体重增加、无法缓解的非放射性背部下腰痛和难治性动脉高血压。体检立即提示库欣综合征。24 小时尿皮质醇证实存在皮质醇增多症,升高的 ACTH 水平提示 ACTH 依赖性库欣综合征。随后,地塞米松抑制试验呈阴性,提示异位 ACTH 分泌。通过影像学和同位素进行病因评估发现右下肺有一个孤立性肺结节,估计为 18 毫米,切除并解剖病理学分析导致了肺类癌肿瘤的诊断,并解决了皮质醇增多症及其并发症。
库欣综合征的诊断延迟会导致相应的发病率和死亡率增加,主要是由于心血管事件。异位库欣综合征的最佳治疗方法是手术切除,因此肿瘤的定位是一个关键因素。