Major hyperestrogenism in a feminizing adrenocortical adenoma despite a moderate overexpression of the aromatase enzyme

• Published in: European journal of endocrinology Vol. 148; no. 4; pp. 457 - 461
• Main Authors: BOURAÏMA, Hélène, LIREUX, Barbara, MITTRE, Hervé, BENHAIM, Annie, HERROU, Michel, MAHOUDEAU, Jacques, GUILLON-METZ, Francoise, KOTTLER, Marie-Laure, REZNIK, Yves
• Format: Journal Article
• Language: English
• Published: Colchester Portland Press 01.04.2003; Oxford Univ. Press
• Subjects: 17-alpha-Hydroxyprogesterone - analysis; Adenoma - complications; Adenoma - enzymology; Adenoma - surgery; Adrenal Cortex - chemistry; Adrenal Cortex Neoplasms - complications; Adrenal Cortex Neoplasms - enzymology; Adrenal Cortex Neoplasms - surgery; Adrenalectomy; Adrenals. Adrenal axis. Renin-angiotensin system (diseases); Adult; Androstenedione - analysis; Aromatase - genetics; Aromatase - metabolism; Biochemistry, Molecular Biology; Biological and medical sciences; Cell Behavior; Cellular Biology; Endocrinopathies; Estradiol - analysis; Estrogens - blood; Estrone - analysis; Feminization - etiology; Gene Expression; Gynecomastia - etiology; Humans; Life Sciences; Male; Malignant tumors; Medical sciences; Molecular biology; Reproductive Biology; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger - analysis; Sexual reproduction; Testosterone - analysis; Endocrinopathy; Case study; Human; Adrenal cortex diseases; Enzyme; Adrenal cortex; Benign neoplasm; Adenoma; Estrogen synthase
• ISSN: 0804-4643; 1479-683X
• DOI: 10.1530/eje.0.1480457

A 30-year-old male was referred for the rapid development of gynecomastia, and dramatic hyperestrogenemia was assessed: plasma estrone, estradiol but also cortisol were not suppressed by high-dose dexamethasone, while gonadotropin pulsatility was completely abolished. A 60-mm right adrenal tumor was evidenced on computed tomography-scan, and the patient underwent adrenalectomy. The tumor was found to express a moderate increase in aromatase activity compared with adjacent non-neoplastic adrenal tissue. Quantitative RT-PCR also showed a weak and non-significant increase in total aromatase mRNA in the tumor compared with normal adrenal tissue. Aromatase transcripts were mainly promoter PII-derived, but different patterns of aromatase minor transcripts were found: promoter I.3- and I.6-derived transcripts were identified in the tumor, while only promoter I.4-derived transcripts were found in normal adrenal. This case report demonstrates that a sharp aromatase overexpression is not a prerequisite for clinical and biochemical hyperestrogenism, and further characterizes the aromatase promoter utilization in this feminizing adrenocortical tumor and in the normal adrenal cortex.