异位ACTH综合征多发生于神经嵴起源的新生物,如肺、胰腺、胸腺、内分泌腺、交感神经系统以及副神经节组织。本文首次报道发生于鼻窦的副神经节瘤一例。患者女性,50岁,因极度衰弱、右侧鼻堵、注意力不集中、记忆力减退及多尿而入院。检查时见患者呈典型Cushing氏综合征外貌,右鼻被黄色息肉样肿块堵塞;血压140/100;实验室检查有血糖过高、血钾过低、碱中毒、血皮质醇过高及血清ACTH水平增高;心电图示U波;X线检查见胸部、脊柱、静脉肾盂造影正常,头颅、鼻窦片示右侧蝶、筛、上颌窦被肿物侵犯,窦腔侧壁及眼眶内壁破坏,断层片示蝶鞍正常;皮质醇水平增高对胃肠外ACTH反应明显,但地塞米松仅能部分抑制;鼻肿物病理检查示部分 Ectopic ACTH syndrome occurs in new organisms of neural crest origin, such as lung, pancreas, thymus, endocrine glands, sympathetic nervous system and paraganglial tissue. This article first reported a case of paraganglioma that occurred in the sinuses. The female patient, 50 years old, was admitted to hospital because of extreme weakness, right nasal obstruction, lack of concentration, memory loss, and polyuria. At the time of examination, the patient presented with a typical appearance of Cushing’s syndrome. The right nose was blocked by a yellow polypoid mass; the blood pressure was 140/100; the laboratory examination was hyperglycemia, hypokalemia, alkalosis, high blood cortisol, and serum ACTH. The level increased; electrocardiogram shows U wave; X-ray examination showed chest, spine, vein pyelography normal, head, sinus shows the right butterfly, ethmoid, maxillary sinus by tumor invasion, sinus cavity sidewall and orbital wall destruction, the fault slice Sphenopalatopharyngosausia is normal; Cortisol levels increase significantly in response to parenteral ACTH, but dexamethasone is only partially inhibited; pathological examination of nasal mass shows part