Virilizing syndrome associated with an adrenal cortical adenoma secreting predominantly testosterone. |
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Authors: | D E Schteingart M C Woodbury H S Tsao A K McKenzie |
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Affiliation: | Ann Arbor, Michigan, USA |
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Abstract: | A 22 year old woman had virilization and mild Cushing's syndrome. Serum testosterone levels were high (3.2 ng/ml), urinary 17-ketosteroid levels slightly elevated (17.5 mg/d) and plasma cortisol at 16:00 hours normal (7.2 μg/dl). Basal level of cortisol secretion was normal but with evidence of autonomy. An abnormal adrenal scan led to an adrenal venogram showing a tumor image in the left adrenal gland. Selective adrenal venous catheterization indicated that the tumor was secreting testosterone and cortisol; left/right adrenal venous values were ng/ml for testosterone and μg/dl for cortisol. Testosterone levels did not rise with human chorionic gonadotropin (hCG). The basal serum level of Δ4 androstenedione was at the upper limit of normal and of 17 α-hydroxyprogesterone high. The patient underwent surgical removal of a well circumscribed, 19 g left adrenal adenoma. Values for serum testosterone, DHEA-S and 17β-estradiol (E2) decreased markedly postoperatively. The in vivo biochemical findings were corroborated in vitro by incubation of tumor tissue slices and nontumorous adrenal cortex. The tumor was capable of secreting testosterone, cortisol, DHEA and E2, but the capacity was greatest for testosterone, DHEA and E2. Analysis of tissue homogenates also revealed the presence of testosterone, cortisol, DHEA and E2. The pattern of steroid production suggests partial 21-hydroxylase deficiency and enhanced 17β-reductase activity. |
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Keywords: | Requests for reprints should be addressed to Dr. David E. Schteingart University Hospital Room S3450 Ann Arbor Michigan 48109. |
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