Virilizing Adrenal Tumor in a Child Suppressed with Dexamethasone for Three Years. Effect of o, p′-DDD on Serum and Urinary Androgens

Abstract
Extensive hormonal evaluation wasperformed in a girl with adrenal carcinomaduringthe primary tumor stage, following adrenalectomy, during the period when metastases were evident and while on treatment with o, p′-DDD. At the age of 14 months a diagnosis of congenital adrenal hyperplasia was made and treatment with dexamethasone (0.125 to 0.25 mg/day) resulted in a fall-off in growth rate, normal advancement in bone age, decrease in virilization and suppression of 17-ketosteroid excretion which continued until 4 3/12 years of age when virilization increased. Atfive years of age elevated serum and urinary androgen levels unsuppressible with dexamethasone were noted. Following removal of a large right adrenal carcinoma, serum and urinary hormone levels returned to normal. Three months following surgery, liver metastases were documented associated with elevated levels of serum androgens. With o, p′- DDD treatment, serum dehydroepiandrosterone sulfat (DS) and urinary 17-ketosteroid (17-KS) excretion fell rapidly while there was a delay in the fallof free androgens. The persistence of free steroid secretion with decreased formation of DS suggests that the o, p′-DDD may have altered sulfatase activity, before causing tumor necrosis and total decrease in steroidogenesis.

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