Female sexual abnormality caused by androgen-producing adrenocortical adenoma： One case report

Abstract

Objective To describe a case of female sexual abnormality with 46, XX caused by an androgen- producing adrenocortical tumor and to explore the mechanism of abnormal androgen secretion from the tumor. Methods The tumor tissues as the experimental group were compared with the normal adrenal tissue. The LH/human chorionic gonadotropin （hCG） receptor was determined by immunohistochemisty, the activity of 3β-hydroxysteroid dehydrogenase （ 3β-HSD ）, 17α-hydroxylase （ CYP17 ）, and 17β-hydroxysteroid oxidoreductase （ 17β-HSD ） by enzyme linked immunosorhent assay （ELISA） and the expression of mRNA of 3 β-HSD2, 17β-HSDB3, CYP17, and LH/hCG receptor by real-quantitative polymerase chain reaction （RQ-PCR）. Results The immunohistochemisty results showed that the LH/hCG receptor was negative in the experiment group, but positive in control. The activity of 3β-HSD and CYP17 of the experiment group was higher than that in the control （ P〈0.01 ） , while the activity of 17β-HSD was lower （2 638. 798 ±70. 551 vs 9 148. 174±382. 836, P〈0.01 ） according to ELISA results. The relative content of 313-HSD2 mRNA of the experiment group was higher than that in the control （ P〈0.05 ） , and the relative content CYP17 mRNA of the experiment group was much higher than that in the control （P〈0.01）. However, the relative eontent of 17β-HSDB3 mRNA and LH/hCG receptor mRNA were much lower than those in the control （ P〈 0.01 ） by RQ-PCR. Conclusion Sexual abnormality and virilization could be caused by the excessive androgen seereted by androgen-producing adrenocortieal tumor, which is an extremely rare disease. The mechanism of the seeretion of androgen from the tumor remains unknown so far. It may be related to the increased activity of 3β-HSD and CYP17, hut has no relationship with the expression of LH/hCG receptor. Key words: Androgen-producing, adrenocortical tumor ; Sexual abnormality ; Virilization