Inhibin A as a tumor marker for primary bilateral macronodular adrenal hyperplasia. (29th April 2020)

• Record Type: Journal Article
• Authors: Wurth, Rachel; Kamilaris, Crystal; Nilubol, Naris; Sadowski, Samira M; Berthon, Annabel; Quezado, Martha M; Faucz, Fabio R; Stratakis, Constantine A; Hannah-Shmouni, Fady
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2020)
• Publication Date: 2020-04-29
• Subjects: Adult -- Female -- Male -- White -- Asian - Korean -- Black - African -- United States; Adrenal -- Adrenal -- Inhibin -- Cortisol -- ACTH -- Macronodular Adrenal Hyperplasia -- Cushing's syndrome -- Macronodular hyperplasia; Hypercortisolaemia -- Weight gain -- Diabetes mellitus type 1 -- Diabetes mellitus type 2 -- Amenorrhoea -- Telangiectasias -- Hyperglycaemia -- Gynaecomastia -- Hypogonadism -- Inhibin A* -- CT scan -- Histopathology -- Molecular genetic analysis -- ACTH -- Immunohistochemistry -- Urinary free cortisol -- 17-hydroxysteroids* -- Adrenalectomy; Genetics; Novel diagnostic procedure -- April -- 2020; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-20-0006
• Languages: English
• ISSNs: 2052-0573

Abstract

Abstract : Summary: Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of ACTH-independent Cushing syndrome (CS). This condition is characterized by glucocorticoid and/or mineralocorticoid excess, and is commonly regulated by aberrant G-protein coupled receptor expression may be subclinical, allowing the disease to progress for years undetected. Inhibin A is a glycoprotein hormone and tumor marker produced by certain endocrine glands including the adrenal cortex, which has not been previously investigated as a potential tumor marker for PBMAH. In the present report, serum inhibin A levels were evaluated in three patients with PBMAH before and after adrenalectomy. In all cases, serum inhibin A was elevated preoperatively and subsequently fell within the normal range after adrenalectomy. Additionally, adrenal tissues stained positive for inhibin A. We conclude that serum inhibin A levels may be a potential tumor marker for PBMAH. Learning points: PBMAH is a rare cause of CS. PBMAH may have an insidious presentation, allowing the disease to progress for years prior to diagnosis. Inhibin A is a heterodimeric glycoprotein hormone expressed in the gonads and adrenal cortex. Inhibin A serum concentrations are elevated in some patients with PBMAH, suggesting the potential use of this hormone as a tumor marker. Further exploration of serum inhibin A concentration, as it relates to PBMAH disease progression, is warranted to determine if this hormone could serve as an early detection marker and/or predictor of successful surgical treatment.