Department of ENT Surgery, University Hospital Ulm, Ulm, Germany.
Laryngoscope. 2013 May;123(5):1132-5. doi: 10.1002/lary.23867. Epub 2013 Jan 8.
Ectopic adrenocorticotropic hormone (ACTH)-secreting tumors account for approximately 10% of Cushing's syndrome (CS). We present an extremely rare case of a patient with CS caused by an ectopic ACTH-secreting pituitary adenoma (EAPA) of the ethmoid sinus. The tumor was identified by positron-emission tomography-computed tomography (PET/CT) using the somatostatin receptor analogue Ga-68-DOTANOC. Transnasal endoscopic resection was performed and the patient showed significant clinical improvement with normalization of the endocrine pituitary axis. Immunostaining showed a somatostatin receptor 2 and 5-positive ACTH-producing adenoma. In patients with ectopic ACTH secretion, Ga-68-DOTANOC-PET/CT may play an important role in the localization of EAPA. Transnasal endoscopic resection is the therapy of choice.
异位促肾上腺皮质激素（ACTH）分泌肿瘤约占库欣综合征（CS）的 10%。我们报告了一例极罕见的由异位 ACTH 分泌性垂体腺瘤（EAPA）引起 CS 的病例，该腺瘤位于筛窦。该肿瘤通过正电子发射断层扫描-计算机断层扫描（PET/CT）使用生长抑素受体类似物 Ga-68-DOTANOC 进行了识别。经鼻内镜切除术，患者的内分泌垂体轴功能正常，临床症状显著改善。免疫组化显示生长抑素受体 2 和 5 阳性的 ACTH 分泌性腺瘤。对于异位 ACTH 分泌患者，Ga-68-DOTANOC-PET/CT 可能在 EAPA 的定位中发挥重要作用。经鼻内镜切除术是首选的治疗方法。
