Ectopic Adrenocorticotropin Syndrome Exhibiting Paradoxical Adrenocorticotropin Responsiveness to Gonadotropin-Releasing Hormone

Endocrine Journal

TAKANOBU YOSHIMOTO³  YASUO TOTSUKA²  REIKA CHO²  TOSHIHIRO IMAKI³  TOSHIO NISHIKAWA¹  KAORU NOMURA³  MAKOTO UJIHARA³  KAZUE TAKANO³ 

[1] Department of Surgical Pathology, Tokyo Women's Medical University;Division of Endocrinology and Metabolism, Kanto Medical Center NTT EC;Department of Medicine, Institute of Clinical Endocrinology, Tokyo Women's Medical University

关键词: Ectopic ACTH syndrome;    GnRH;    Carcinoid;   

DOI  :  10.1507/endocrj.48.19

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(11)Cited-By(2)In a 37-year-old man who had Cushing's syndrome, investigations, including overnight dexamethasone suppression test, corticotropin-releasing hormone (CRH) test, pituitary MRI and inferior petrosal sinus sampling suggested the presence of ectopic adrenocorticotropin (ACTH) production. Interestingly, gonadotropin-releasing hormone (GnRH) increased plasma ACTH from 73pg/ml to 708pg/ml at 15min. Furthermore, desmopressin also increased plasma ACTH whereas CRH and thyrotropin-releasing hormone (TRH) had no effect. Such paradoxical responses of plasma ACTH were observed repeatedly. A thoracic CT scan revealed a right anterior mediastinal mass, which was surgically resected. Histological and immunohistochemical examination confirmed that the tumor was an ACTH-producing carcinoid. ACTH and cortisol decreased immediately following surgery. Neither desmopressin nor GnRH administration resulted in elevation of plasma ACTH while ACTH-responsiveness to dexamethasone and CRH was restored. To our knowledge, this is the first report documenting GnRH responsiveness in ectopic ACTH syndrome.

【 授权许可】

Unknown