title: Major hyperestrogenism in a feminizing adrenocortical adenoma despite a moderate overexpression of the aromatase enzyme.
abstract: 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.
authors.affiliation: EA 2608-USC INRA, Université de Caen, 14032 Caen Cedex, France.
authors.forename: Helene
authors.initials: H
authors.lastname: Bouraïma
authors.forename: Barbara
authors.initials: B
authors.lastname: Lireux
authors.forename: Hervé
authors.initials: H
authors.lastname: Mittre
authors.forename: Annie
authors.initials: A
authors.lastname: Benhaim
authors.forename: Michel
authors.initials: M
authors.lastname: Herrou
authors.forename: Jacques
authors.initials: J
authors.lastname: Mahoudeau
authors.forename: Francoise
authors.initials: F
authors.lastname: Guillon-Metz
authors.forename: Marie-Laure
authors.initials: ML
authors.lastname: Kottler
authors.forename: Yves
authors.initials: Y
authors.lastname: Reznik
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000032:analysis; D000236:Adenoma / Q000150:complications* / Q000201:enzymology / Q000601:surgery; D000302:Adrenal Cortex / Q000737:chemistry; D000306:Adrenal Cortex Neoplasms / Q000150:complications* / Q000201:enzymology / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D000735:Androstenedione / Q000032:analysis; D001141:Aromatase / Q000235:genetics* / Q000378:metabolism; D004958:Estradiol / Q000032:analysis; D004967:Estrogens / Q000097:blood*; D004970:Estrone / Q000032:analysis; D005262:Feminization / Q000209:etiology*; D015870:Gene Expression; D006177:Gynecomastia / Q000209:etiology; D006801:Humans; D008297:Male; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013739:Testosterone / Q000032:analysis
chemical_list: D004967:Estrogens; D012333:RNA, Messenger; D004970:Estrone; D013739:Testosterone; D000735:Androstenedione; D004958:Estradiol; D019326:17-alpha-Hydroxyprogesterone; D001141:Aromatase