Ectopic Cushing's syndrome due to retroperitoneal ACTH-producing paragangliomas

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...

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Bibliographic Details
Published in:Canadian Urological Association journal 2016-09, Vol.10 (9-10), p.E320-E323
Main Authors: 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
Format: Article
Language:English
Subjects:
ACTH
Care and treatment
Case Report
Case studies
Complications and side effects
Cushing syndrome
Diagnosis
Gliomas
Health aspects
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Summary: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.
ISSN:1911-6470
1920-1214
1920-1214
DOI:10.5489/cuaj.3153