title: Virilizing adrenal adenoma in an adult with the Beckwith-Wiedemann syndrome: paradoxical response to dexamethasone.
abstract: An adult woman with Beckwith-Wiedemann syndrome, hemihypertrophy and an androgen-secreting adrenal adenoma is described. She presented with a 7-year history of progressive virilization and was found to have high plasma levels of testosterone and dehydroepiandrosterone (DHEA) sulphate and elevated levels of urinary metabolites of testosterone and its precursors. Administration of dexamethasone was associated with progressive rises in plasma 17 alpha OH progesterone, 11 beta-desoxycortisol, DHEA sulphate, androstenedione and testosterone, together with increased urinary excretion of androsterone, 11 beta OH androsterone, etiocholanolone, DHEA, and 16 alpha OH DHEA. Hormone levels fell to normal following removal of the tumour.
authors.affiliation: Department of Endocrinology, Royal Brisbane Hospital, Australia.
authors.forename: W M
authors.initials: WM
authors.lastname: Clouston
authors.forename: G C
authors.initials: GC
authors.lastname: Cannell
authors.forename: B G
authors.initials: BG
authors.lastname: Fryar
authors.forename: J W
authors.initials: JW
authors.lastname: Searle
authors.forename: N I
authors.initials: NI
authors.lastname: Martin
authors.forename: R H
authors.initials: RH
authors.lastname: Mortimer
mesh_terms: D019326:17-alpha-Hydroxyprogesterone; D000236:Adenoma / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D000728:Androgens / Q000652:urine; D000735:Androstenedione / Q000097:blood; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications* / Q000188:drug therapy; D003350:Cortodoxone / Q000097:blood; D003687:Dehydroepiandrosterone / Q000031:analogs & derivatives / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate; D003907:Dexamethasone; D005260:Female; D006801:Humans; D006908:Hydroxyprogesterones / Q000097:blood; D008875:Middle Aged; D013739:Testosterone / Q000097:blood / Q000652:urine; D014770:Virilism
chemical_list: D000728:Androgens; D006908:Hydroxyprogesterones; D013739:Testosterone; D000735:Androstenedione; D003687:Dehydroepiandrosterone; D019314:Dehydroepiandrosterone Sulfate; D019326:17-alpha-Hydroxyprogesterone; D003907:Dexamethasone; D003350:Cortodoxone