Virilizing adrenal tumor in a child suppressed with dexamethasone for three years. Effect of o,p'-DDD on serum and urinary androgens.
Extensive hormonal evaluation was performed in a girl with adrenal carcinoma during the 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. At five 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. There months following surgery, liver metastases were documented associated with elevated levels of serum androgens. With o,p'-DDD treatment, serum dehydroepiandrosterone sulfate (DS) and urinary 17-ketosteroid (17-KS) excretion fell rapidly while there was a delay in the fall of 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.
D015068:17-Ketosteroids / Q000652:urine; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D000308:Adrenocortical Hyperfunction / Q000175:diagnosis / Q000188:drug therapy; D000728:Androgens / Q000378:metabolism*; D002675:Child, Preschool; D003687:Dehydroepiandrosterone / Q000097:blood; D003864:Depression, Chemical; D003907:Dexamethasone / Q000494:pharmacology* / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D008939:Mitotane / Q000009:adverse effects* / Q000627:therapeutic use; D009362:Neoplasm Metastasis; D014770:Virilism / Q000209:etiology*
D015068:17-Ketosteroids; D000728:Androgens; D003687:Dehydroepiandrosterone; D008939:Mitotane; D003907:Dexamethasone