Meinardi J R, van den Berg G, Wolffenbuttel B H R, Kema I P, Dullaart R P F
Department of Endocrinology, University Medical Centre Groningen, Groningen, the Netherlands.
Neth J Med. 2006 Jan;64(1):23-7.
A 43-year-old man presented with fluctuating symptoms of weight gain, shortness of breath, pretibial oedema, associated with anxiety and memory disturbances. Laboratory investigation revealed an adrenocorticotropin (ACTH)-dependent cyclical Cushing's syndrome characterised by remarkable variations in urinary cortisol excretions ranging from 27 to 28,050 nmol/ 24 h. Magnetic resonance imaging (MRI ) of the pituitary was normal and ectopic ACTH production was suspected. A tumour in the right anterior mediastinum was revealed on octreotide receptor scintigraphy, which had initially been overlooked on computed tomography (CT) scanning. A thymic carcinoid tumour was suspected, which was supported by increased levels of urinary serotonin, while platelet serotonin and urinary 5-hydroxyindoleacetic acid levels were normal. The tumour was removed surgically and histological examination revealed an atypical thymic carcinoid tumour. Postoperatively, the patient's symptoms disappeared rapidly. He underwent external radiotherapy and is still free of symptoms after almost two years of follow-up. For clinical practice, a cyclical Cushing's syndrome should be suspected in any patient with clinical signs of Cushing's syndrome but normal biochemistry. Repeated measurement of urinary cortisol excretion is then required to establish or rule out the diagnosis.
一名43岁男性出现体重增加、呼吸急促、胫前水肿等症状波动,并伴有焦虑和记忆障碍。实验室检查发现一种促肾上腺皮质激素(ACTH)依赖性周期性库欣综合征,其特征为尿皮质醇排泄量显著变化,范围从27至28,050 nmol/24小时。垂体磁共振成像(MRI)正常,怀疑有异位ACTH产生。奥曲肽受体闪烁扫描显示右前纵隔有一个肿瘤,该肿瘤最初在计算机断层扫描(CT)中被漏诊。怀疑是胸腺类癌肿瘤,尿5-羟色胺水平升高支持这一诊断,而血小板5-羟色胺和尿5-羟吲哚乙酸水平正常。肿瘤通过手术切除,组织学检查显示为非典型胸腺类癌肿瘤。术后,患者症状迅速消失。他接受了外照射放疗,经过近两年的随访仍无症状。在临床实践中,对于任何有库欣综合征临床体征但生化指标正常的患者,都应怀疑周期性库欣综合征。然后需要反复测量尿皮质醇排泄量以确立或排除诊断。