Ectopic adrenocorticotropic hormone syndrome originating from retroperitoneal paraganglioma: one case report and literature review
10.3760/cma.j.cn431274-20220307-00166
- VernacularTitle:腹膜后副神经节瘤致异位ACTH综合征一例报告并文献复习
- Author:
Ying LE
1
;
Ying ZHANG
;
Yan WU
;
Yanfeng LIU
;
Quanzhou PENG
;
Libo CHEN
;
Yuanyuan HU
;
Qingmei ZHANG
Author Information
1. 深圳市人民医院(暨南大学第二临床医学院，南方科技大学第一附属医院)内分泌科，深圳 518020
- Keywords:
ACTH syndrome, ectopic;
Paraganglioma;
Cushing syndrome
- From:
Journal of Chinese Physician
2022;24(10):1498-1503
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To present the clinical characteristics and treatment on patients with ectopic adrenocorticotropic hormone(ACTH) syndrome (EAS) caused by the retroperitoneal paraganglioma.Methods:The clinical data of a case of EAS caused by retroperitoneal paraganglioma were analyzed retrospectively, and the related literature at home and abroad was reviewed.Results:The 53-year-old female patient was admitted to endocrinology department due to a fifteen-year history of hypertension, accompanied by fatigue for three months, headache and dizziness for one month. The laboratory data demonstrated severe hypokalemia, high level of serum and urinary cortisol, while the ACTH level remained unsuppressed. The 24 h urinary vanillyl mandelic acid (VMA) and serum free methoxyepinephrine (MNs) level were elevated. The abdominal computed tomographic scan suggested a retroperitoneal mass next to the abdominal aorta. After the retroperitoneal tumor resection was performed, immunohistochemical staining of the tumor revealed Syn (+ ), CgA (+ ), ACTH (focal + ). By the retrospective analysis of 22 similar cases from 16 papers and the case summarized above, we found that most patients with EAS caused by the paraganglioma could demonstrate the typical clinical features of Cushing′s syndrome, while lack of the manifestation of paraganglioma. Therefore, preoperative preparations for paraganglioma were usually neglected.Conclusions:Ectopic ACTH syndrome (EAS) originating from paraganglioma is very rare. To improve the diagnosis rate, examination for catecholamine, MNs and 24 h urinary VMA before surgery in patients with EAS is suggested. Considering surgical resection as the optimal treatment, comprehensive preoperative preparations for both paraganglioma and Cushing′s syndrome are significant. A genetic test for pheochromocytoma/ paraganglioma and lifelong postoperative follow-up are also recommend.
