Pancreatic ACTH Hypersecretion and Pituitary Macroadenoma. Issue 1 (1st February 2023)

• Record Type: Journal Article
• Authors: Bettale, Chiara M; Allen, Jason W; Mahdi, Zaid K; Ioachimescu, Adriana G
• Is Part Of: JCEM case reports. Volume 1:Issue 1(2023)
• Publication Date: 2023-02-01
• Subjects: Cushing syndrome -- ectopic ACTH syndrome -- neuroendocrine tumor -- pancreatic neuroendocrine neoplasm -- pituitary adenoma; 616.409
• DOI: 10.1210/jcemcr/luad007
• Languages: English
• ISSNs: 2755-1520

Abstract

Abstract: A 55-year-old woman admitted for hypertensive emergency and myocardial infarction reported weight gain, muscle weakness, easy bruising, and recent-onset diabetes in the past 3 to 12 months. Urinary and salivary cortisol and adrenocorticotropin hormone (ACTH) levels were elevated. Pituitary imaging detected a macroadenoma. ACTH and cortisol did not increase after corticotropin-releasing hormone administration. Imaging revealed a large pancreatic mass. Pathology indicated a well-differentiated World Health Organization (WHO) grade 2 distal pancreatic neuroendocrine neoplasm which stained for ACTH by immunohistochemistry. Postoperatively, Cushing manifestations resolved, ACTH and cortisol levels became low, and patient required hydrocortisone replacement for 7 months. During the 3.5 years of follow-up, the pituitary macroadenoma size remained stable and pituitary hormone axes other than ACTH remained normal. This extremely rare case of ectopic ACTH-secreting pancreatic neuroendocrine tumor coexisting with a nonfunctioning pituitary macroadenoma illustrates the importance of dynamic endocrine testing in Cushing syndrome.