Chen Fan, Wang Xiangyu, Wang Yang, Meng Hui, Hou Xinguo, Zhu Yaofeng, Gao Wei, Jiang Xuewen, Chen Shouzhen, Zhang Zhaocun, Zou Zhichuan, He Tianyi, Yang Yue, Zhu Kejia, Wang Yong, Liu Yaxiao, Cui Jianfeng, Shi Benkang, Yin Gang
Department of Urology, Qilu Hospital, Shandong University, Jinan, Shandong, China;; College of Medicine, University of Kentucky, Lexington, KY, United States.
Department of Urology, The People's Hospital of Liaocheng, Liaocheng, Shandong, China.
Can Urol Assoc J. 2016 Sep-Oct;10(9-10):E320-E323. doi: 10.5489/cuaj.3153. Epub 2016 Sep 13.
Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH). He got clinical and biochemical recoveries without any recurrent evidence at the nine-month followup.
肾上腺外嗜铬细胞瘤,即副神经节瘤,是一种源于肾上腺外嗜铬细胞的罕见肿瘤。由副神经节瘤引起的库欣综合征(CS)极为罕见。我们报告一例53岁男性患者,有高血压、糖尿病及低钾血症症状。计算机断层扫描(CT)显示腹膜后有两个肿块及双侧肾上腺增生。结合实验室检查,高度怀疑是由多发副神经节瘤引起的异位性CS。患者接受了腹膜后肿瘤、左肾及左肾上腺切除术;术后组织病理学证实为两个副神经节瘤,两者促肾上腺皮质激素(ACTH)染色均为阳性。在9个月的随访中,患者临床及生化指标恢复正常,无任何复发迹象。