Disorganized Steroidogenesis in Adrenocortical Carcinoma, a Case Study
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Disorganized Steroidogenesis in Adrenocortical Carcinoma, a Case Study Toyoyoshi Uchida 1 & Koshiro Nishimoto 2,3 & Yuki Fukumura 4 & Miki Asahina 4 & Hiromasa Goto 1 & Yui Kawano 1 & Fumitaka Shimizu 5 & Akira Tsujimura 5 & Tsugio Seki 6 & Kuniaki Mukai 3,7 & Yasuaki Kabe 3 & Makoto Suematsu 3 & Celso E. Gomez-Sanchez 8 & Takashi Yao 4 & Shigeo Horie 5 & Hirotaka Watada 1
# Springer Science+Business Media New York 2016
Abstract Most adrenocortical carcinomas (ACCs) produce excessive amounts of steroid hormones including aldosterone, cortisol, and steroid precursors. However, aldosterone- and cortisol-producing cells in ACCs have not yet been immunohistochemically described. We present a case of ACC causing mild primary aldosteronism and subclinical Cushing’s syndrome. Removal of the tumor cured both conditions. In order to examine the expression patterns of the steroidogenic enzymes responsible for adrenocortical hormone production, 10 tumor portions were immunohistochemically analyzed for aldosterone synthase (CYP11B2), 11βhydroxylase (CYP11B1, cortisol-synthesizing enzyme), 3β-hydroxysteroid dehydrogenase (3βHSD, upstream enzyme for both CYP11B2 and CYP11B1), and 17αhydroxylase/C17-20 lyase (CYP17, upstream enzyme for CYP11B1, but not for CYP11B1). CYP11B2, CYP11B1, and 3βHSD were expressed sporadically,
and their expression patterns varied significantly among the different tumor portions examined. The expression of these enzymes was random and not associated with each other. CYP17 was expressed throughout the tumor, even in CYP11B2-positive cells. Small tumor cell populations were aldosterone- or cortisol-producing cells, as judged by 3β HSD coinciding w ith either CYP11B2 or CYP11B1, respectively. These results suggest that the tumor produced limited amounts of aldosterone and cortisol due to the lack of the coordinated expression of steroidogenic enzymes, which led to mild clinical expression in this case. We delineated the expression patterns of steroidogenic enzymes in ACC. The coordinated expression of steroidogenic enzymes in normal and adenoma cells was disturbed in ACC cells, resulting in the inefficient production of steroid hormones in relation to the large tumor volume.
Toyoyoshi Uchida and Koshiro Nishimoto contributed equally to this work. * Toyoyoshi Uchida [email protected]
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Department of Human Pathology, Juntendo University, Graduate School, Tokyo 113-8421, Japan
* Koshiro Nishimoto [email protected]
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Department of Urology, Juntendo University, Graduate School, Tokyo 113-8421, Japan
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Department of Medical Education, College of Medicine, California University of Science and Medicine, 1405 West Valley Blvd #101, Colton, CA 92324, USA
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Departments of Metabolism & Endocrinology, Juntendo University, Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan
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Department of Uro-Oncology, Saitama Medical University International Medical Center, Hidaka, Japan
Medical Education Center, School of Medicine, Keio University, Tokyo 160-8582, Japan
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Endocrino
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