PEDIATRICS Vol. 68 No. 2 August 1981, pp. 242-246
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Adrenocortical Tumor in a Patient with Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

Songja Pang MD1, Dorothy Becker MD1, James Cotelingam MD1, Thomas P. Foley Jr MD1, and Allan L. Drash MD1

1 Department of Pediatrics and Pathology, School of Medicine, University of Pittsburgh and Children's Hospital of Pittsburgh, Pittsburgh

An adrenal cortical tissue tumor developed in a patient with poorly controlled salt-losing congenital adrenal hyperplasia. A 16-year-old girl became progressively virilized from 13 to 16 years of age. Base line serum progesterone, 17-hydroxyprogesterone, and testosterone levels were high and there was a diurnal pattern of the hormones. Initially elevated urinary 17-ketosteroid and serum steroid levels were decreased by high dose dexamethasone therapy, and at laparotomy an adenoma was found in the cortex of the hyperplastic left adrenal gland. It is inferred that persistent adrenocorticotropic hormone stimulation may result in neoplastic transformation of hyperplastic adrenal cortical tissue in patients with congenital adrenal hyperplasia.

Submitted on June 18, 1980
Accepted on November 21, 1980




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