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

Department of Pediatrics, Division of Pediatric Endocrinology, New York Hospital-Cornell Medical Center 525 East 68th Street, New York, N. Y. 10021
Department of Pathology, Overlook Hospital Summit,N. J. 07901,
College of Physicians and Surgeons, Columbia University N.Y.

Reprint address: Maria I. New, M. D., The New York Hospital-Cornell Medical Center, 525 East 68th Street, New York, N.Y. 10021.

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.

Supported in part by grant awards: USPHS, NIH, HD 00072; USPHS, NIH, Division of Research Facilities and Resources, Pediatric Clinical Research Center, RR 47; National Foundation-March of Dimes CRBS 278; USPHS, NIH Research Fellowship AM 00329; and by Deutsche Forschungsgemeinschaft.

Received July 8, 1975.

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