VIRILIZING ADRENAL ADENOMA IN AN ADULT WITH THE BECKWITH-WIEDEMANN SYNDROME: PARADOXICAL RESPONSE TO DEXAMETHASONE

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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.