Abstract: Background　Ectopic adrenocorticotropic hormone syndrome（EAS） refers to a series of clinical syndromes caused by the secretion of adrenocorticotropic hormone（ACTH） or other proopiomelanocortin （POMC） derived peptide by tissues outside the pituitary gland，which stimulates adrenocortical hyperplasia and makes it secrete excessive glucocorticoid.EAS caused by pheochromocytoma is very rare.Objective　To describe the clinical features of a case of pheochromocytoma combined familial adenomatous polyposis（FAP） with EAS，and to investigate its diagnosis and treatment approaches.Methods　The patient was admitted to Sichuan Provincial People's Hospital on June 12，2014 because of "recurrent consciousness disorder，fatigue，headache，palpitation，excessive sweating and insomnia for one month".The patient was diagnosed with pheochromocytoma combined FAP with EAS，and the clinical data of this patient were retrospectively reviewed and the literature was reviewed.Results　The patient had typical manifestations of pheochromocytoma such as paroxysmal headache，palpitations，blurred vision，excessive sweating and large blood pressure fluctuations，accompanied by clinical manifestations of EAS such as hyperglycemia，fatigue，insomnia，and ematiation，while the early clinical manifestations of FAP had been hidden.Conclusion　EAS has a variety of clinical manifestations and is easily misdiagnosed and mistreated.When the patients experience clinically the severe hypertension，hypokalemia，arrhythmias and secondary diabetes，the Cushing's syndrome should be considered，especially the possibility of EAS.It requires a combination of clinical features，biochemical and hormone secretion，imaging，and pathology results for a comprehensive judgment.Early clinical manifestations of FAP are non-specific，the family investigation and colonoscopy screening for populations at risk are important ways to detect patients with FAP early.

Key words: Pheochromocytoma, Ectopic adrenocorticotropic hormone syndrome, Adenomatous polyps, Cushing syndrome, Case reports, Historical article

摘要： 背景　异位促肾上腺皮质激素综合征（EAS）是指垂体以外的组织分泌促肾上腺皮质激素（ACTH）或其他阿片黑素促皮质素原（POMC）衍生肽，刺激肾上腺皮质增生，使之分泌过量糖皮质激素，从而引起的一系列临床综合征。嗜铬细胞瘤导致的EAS非常罕见。目的　报道1例嗜铬细胞瘤合并家族性腺瘤性息肉病致EAS的临床特点，探讨其诊治。方法　患者因“反复意识障碍，乏力，头痛，心悸，多汗，失眠1个月”于2014-06-12入住四川省人民医院，诊断为嗜铬细胞瘤合并家族性腺瘤性息肉病致EAS，回顾性分析患者的临床资料并复习文献。结果　患者具有阵发性头痛、心悸、视物模糊、多汗及血压波动大等嗜铬细胞瘤的典型表现，同时伴高血糖、乏力、失眠、消瘦等EAS的临床表现。而家族性腺瘤性息肉病的早期临床表现隐匿。结论　EAS临床表现多样，易误诊误治。临床遇到严重高血压、低血钾、心律失常及继发性糖尿病患者，应考虑库欣综合征尤其EAS可能。需结合临床特点、生化及激素分泌、影像学、病理学进行综合判断。家族性腺瘤性息肉病的早期临床表现无特异性，家系调查和对高危人群进行肠镜筛查是发现该病早期患者的重要途径。

关键词: 嗜铬细胞瘤, 异位促肾上腺皮质激素综合征, 腺瘤性息肉, 库欣综合征, 病例报告, 历史文献