Increased 3-hydroxysteroid dehydrogenase and 17-hydroxylase activities in a virilized pubertal Chinese girl with adrenal adenoma

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 498-523-Female Reproductive Endocrinology & Case Reports
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-521
Yang Guoqing*, Dou Jing TAO, Zhang Xiaoling, Lv Zhaohui, Mu Yiming and Lu Juming
Chinese PLA General Hospital, Beijing, China
Objective To report a case of 46, XX female pseudohermaphrodism caused by an androgen-producing adrenocortical tumor, and to explore the mechanism of androgen secretion from this tumor. Methods Compared between two groups: tumor tissues as experimental group, and normal adrenal tissues as controls, and measured LH/hCG receptor by immunohistochemisty, the activities of 3β- hydroxysteroid dehydrogenase, 17α- hydroxylase and 17β- hydroxysteroid oxidoreductase by enzyme linked immunosorbent assay (ELISA), and the expressions of mRNA of 3β-HSD2, 17β-HSDB3, CYP17 and LH/hCG receptor by real-quantitative polymerase chain reaction ( RQ-PCR). Results It is showed that the LH/hCG receptor was negative in the experimental group and positive in the control group. The activities of 3β- hydroxysteroid dehydrogenase and 17α- hydroxylase in the experimental group were higher than those of the controls (p<0.01), but the activity of 17β- hydroxysteroid oxidoreductase was lower (p<0.01), compared with that in the controls. Both the relative mRNA contents of 3β-HSD2 and CYP17 in the experimental group were higher than those of the controls (p<0.05 and p<0.01 respectively). The relative mRNA contents of 17β-HSDB3 and LH/hCG receptor were much lower (p<0.01), though. Conclusion We are reporting a case of virilization caused by excess androgen secretion from androgen-producing adrenocortical tumor, which is extremely rare. So far, the mechanism remains unclear. Our study demonstrated that it may be related to the increased activities of 3β- hydroxysteroid dehydrogenase and 17α- hydroxylase, but has no relationship with the expression of LH/hCG receptor.

[1] Nakagawa Y, Hirao Y, Tsujimoto S, et al. [A case of infantile virilizing adrenocortical tumor]. Hinyokika Kiyo, 1989, 35 (10): 1731-1736    [2]      Deng Y, Osamura Y, Tanaka M, et al. A case of testosterone-secreting adrenal cortical adenoma with spironolactone body-like inclusion. Acta Pathol Jpn, 1990, 40 (1): 67-72    [3]      Pabon J E, Li X, Lei Z M, et al. Novel presence of luteinizing hormone/chorionic gonadotropin receptors in human adrenal glands. J Clin Endocrinol Metab, 1996, 81 (6): 2397-2400    [4]      Carlson H E. Human adrenal cortex hyperfunction due to LH/hCG. Mol Cell Endocrinol, 2007, 269 (1-2): 46-50  [5]      Saner-Amigh K, Mayhew B A, Mantero F, et al. Elevated expression of luteinizing hormone receptor in aldosterone-producing adenomas. J Clin Endocrinol Metab, 2006, 91 (3): 1136-1142

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