Increased 3β‑hydroxysteroid dehydrogenase 2 and 17α‑hydroxylase activities in a virilized adolescent female with adrenal adenoma: A case report

Yang,Guoqing; Dou,Jingtao; Zhang,Xiaolin; Gu,Weijun; Lv,Zhaohui; Du,Jin; Ba,Jianming; Mu,Yiming; Lu,Juming

doi:10.3892/etm.2015.2935

Increased 3β‑hydroxysteroid dehydrogenase 2 and 17α‑hydroxylase activities in a virilized adolescent female with adrenal adenoma: A case report

Authors:
- Guoqing Yang
- Jingtao Dou
- Xiaolin Zhang
- Weijun Gu
- Zhaohui Lv
- Jin Du
- Jianming Ba
- Yiming Mu
- Juming Lu
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Affiliations: Department of Endocrinology, PLA General Hospital, Beijing 100853, P.R. China
Published online on: December 14, 2015 https://doi.org/10.3892/etm.2015.2935
Pages: 530-534

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Abstract

In the present study, the case of a female patient with pseudo‑hermaphrodism caused by an androgen‑producing adrenocortical tumor is presented, and the possible mechanism is investigated. The expression of the luteinizing hormone/human chorionic gonadotrophin (LH/hCG) receptor in tumor tissues and normal adrenal tissues was analyzed using immunohistochemistry. Furthermore, the activities of 3β‑hydroxysteroid dehydrogenase 2 (HSD2), cytochrome P450 17α‑hydroxylase (CYP17) and 17β‑hydroxysteroid dehydrogenase 3 (HSD3) enzymes were measured using enzyme‑linked immunosorbent assay, and the expression levels of 3β‑HSD2, 17β‑HSD3, CYP17 and LH/hCG receptor mRNA were determined by quantitative polymerase chain reaction (qPCR). Immunohistochemical staining for the LH/hCG receptor was negative in the tumor tissue and positive in the normal adrenal tissue. The activities of 3β‑HSD2 and CYP17 in the tumor tissue were higher than those in the normal tissue (P<0.01), whereas the activity of 17β‑HSD3 was lower (P<0.01). The mRNA levels of 3β‑HSD2 and CYP17 were higher (P<0.01) and the levels of 17β‑HSD3 and LH/hCG receptor were lower (P<0.01) in the tumor tissue compared with those of the normal tissue. In conclusion, in the present study, a rare case of virilization by an androgen‑producing adrenocortical tumor is present. The results indicate that it may be associated with increased activities of 3β‑HSD2 and CYP17 but not with the expression of the LH/hCG receptor.

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1	Rohana AG, Ming W, Norlela S and Norazmi MK: Functioning adrenal adenoma in association with congenital adrenal hyperplasia. Med J Malaysia. 62:158–159. 2007.PubMed/NCBI
2	Bédrine H D.M..Mangeot JP and Bégueri F: Familial male pseudo-hermaphrodism of karyotype XY and ambiguous morphology. Gynecol Obstet (Paris). 67:15–28. 1968.(In French). PubMed/NCBI
3	Ben Charfeddine I, Riepe FG, Kahloul N, Kulle AE, Adala L, Mamaï O, Amara A, Mili A, Amri F, Saad A, Holterhus PM and Gribaa M: Two novel CYP11B1 mutations in congenital adrenal hyperplasia due to steroid 11β hydroxylase deficiency in a Tunisian family. Gen Comp Endocrinol. 175:514–518. 2012. View Article : Google Scholar : PubMed/NCBI
4	Choukair D, Beuschlein F, Zwermann O, Wudy SA, Haufe S, Holland-Cunz S and Bettendorf M: Virilization of a young girl caused by concomitant ectopic and intra-adrenal adenomas of the adrenal cortex. Horm Res Paediatr. 79:318–322. 2013. View Article : Google Scholar : PubMed/NCBI
5	Nakamura A, Schimizu C, Nagai S, Taniguchi S, Umetsu M, Atsumi T, Wada N, Yoshioka N, Ono Y, Sasano H and Koike T: Unilateral adrenalectomy improves insulin resistance and polycystic ovaries in a middle-aged woman with virilizing adrenocortical adenoma complicated with Cushing's syndrome. J Endocrinol Invest. 30:65–69. 2007. View Article : Google Scholar : PubMed/NCBI
6	Wang S, Kamat A, Pergola P, Swamy A, Tio F and Cusi K: Metabolic factors in the development of hepatic steatosis and altered mitochondrial gene expression in vivo. Metabolism. 60:1090–1099. 2011. View Article : Google Scholar : PubMed/NCBI
7	Tanaka S, Tanabe A, Aiba M, Hizuka N, Takano K, Zhang J and Young WF: Glucocorticoid- and androgen-secreting black adrenocortical adenomas, unique cause of corticotropin-independent Cushing syndrome. Endocr Pract. 17:e73–e78. 2011. View Article : Google Scholar : PubMed/NCBI
8	Cordera F, Grant C, van Heerden J, Thompson G and Young W: Androgen-secreting adrenal tumors. Surgery. 134:874–880. 2003. View Article : Google Scholar : PubMed/NCBI
9	Lipsett MB, Hertz R and Ross GT: Clinical and pathophysiologic aspects of adrenocortical carcinoma. Am J Med. 35:374–383. 1963. View Article : Google Scholar : PubMed/NCBI
10	Fukushima DK and Gallagher TF: Steroid Production in ‘Nonfunctioning’ Adrenal Cortical Tumor: J Clin Endocrinol Metab. 23:923–927. 1963.
11	Kim BY, Chun AR, Kim KJ, Jung CH, Kang SK, Mok JO and Kim CH: Clinical characteristics and metabolic features of patients with adrenal incidentalomas with or without subclinical Cushin's syndrome. Endocrinol Metab (Seoul). 29:457–463. 2014. View Article : Google Scholar : PubMed/NCBI
12	Rodríguez-Gutiérrez R, Bautista-Medina MA, Teniente-Sanchez AE, Zapata-Rivera MA and Montes-Villarreal J: Pure androgen-secreting adrenal adenoma associated with resistant hypertension. Case Rep Endocrinol. 2013:3560862013.PubMed/NCBI
13	Petersons CJ and Burt MG: The utility of adrenal and ovarian venous sampling in the investigation of androgen-secreting tumours. Intern Med J. 41:69–70. 2011. View Article : Google Scholar : PubMed/NCBI
14	Nguyen PT, Lee RS, Conley AJ, Sneyd J and Soboleva TK: Variation in 3β-hydroxysteroid dehydrogenase activity and in pregnenolone supply rate can paradoxically alter androstenedione synthesis. J Steroid Biochem Mol Biol. 128:12–20. 2012. View Article : Google Scholar : PubMed/NCBI
15	de Groot JW, Links TP, Themmen AP, Looijenga LH, de Krijger RR, van Koetsveld PM, Hofland J, van den Berg G, Hofland LJ and Feelders RA: Aberrant expression of multiple hormone receptors in ACTH-independent macronodular adrenal hyperplasia causing Cushing's syndrome. Eur J Endocrinol. 163:293–299. 2010. View Article : Google Scholar : PubMed/NCBI
16	Rask E, Schvarcz E, Hellman P, Hennings J, Karlsson FA and Rao CV: Adrenocorticotropin-independent Cushing's syndrome in pregnancy related to overexpression of adrenal luteinizing hormone/human chorionic gonadotropin receptors. J Endocrinol Invest. 32:313–316. 2009. View Article : Google Scholar : PubMed/NCBI
17	Morris LF, Park S, Daskivich T, Churchill BM, Rao CV, Lei Z, Martinez DS and Yeh MW: Virilization of a female infant by a maternal adrenocortical carcinoma. Endocr Pract. 17:e26–e31. 2011. View Article : Google Scholar : PubMed/NCBI
18	Fujieda K and Ito Y: Inactivating and activating mutations of the human LH/hCG receptor leading to male pseudohermaphroditism and familial male-limited precocious puberty. Nihon Rinsho. 60:265–271. 2002.(In Japanese). PubMed/NCBI
19	Lai KP, Huang CK, Fang LY, Izumi K, Lo CW, Wood R, Kindblom J, Yeh S and Chang C: Targeting stromal androgen receptor suppresses prolactin-driven benign prostatic hyperplasia (BPH). Mol Endocrinol. 27:1617–1631. 2013. View Article : Google Scholar : PubMed/NCBI
20	Mieritz MG, Sorensen K, Aksglaede L, Mouritsen A, Hagen CP, Hilsted L, Andersson AM and Juul A: Elevated serum IGF-I, but unaltered sex steroid levels, in healthy boys with pubertal gynaecomastia. Clin Endocrinol (Oxf). 80:691–698. 2014. View Article : Google Scholar : PubMed/NCBI
21	Monroe WE, Panciera D and Zimmerman KL: Concentrations of noncortisol adrenal steroids in response to ACTH in dogs with adrenal-dependent hyperadrenocorticism, pituitary-dependent hyperadrenocorticism, and nonadrenal illness. J Vet Intern Med. 26:945–952. 2012. View Article : Google Scholar : PubMed/NCBI