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Case Report

Familial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study

by
Nae Takizawa
1,†,
Susumu Tanaka
2,†,
Koshiro Nishimoto
3,*,†,
Yuki Sugiura
4,
Makoto Suematsu
4,
Chisato Ohe
5,
Haruyuki Ohsugi
1,
Yosuke Mizuno
6,
Kuniaki Mukai
4,
Tsugio Seki
7,
Kenji Oki
8,
Celso E. Gomez-Sanchez
9 and
Tadashi Matsuda
1
1
Department of Urology and Andrology, Kansai Medical University, Osaka 573-1191, Japan
2
Department of Anatomy, Kansai Medical University, Osaka 573-1191, Japan
3
Department of Uro-Oncology, Saitama Medical University, International Medical Center 1397-1 Yamane, Hidaka 350-1298, Japan
4
Departments of Biochemistry, School of Medicine, Keio University, Tokyo 160-8582, Japan
5
Department of Pathology, Kansai Medical University, Osaka 573-1191, Japan
6
Biomedical Research Center, Division of Morphological Science, Saitama Medical University, Saitama 350-0495, Japan
7
Department of Medical Education, California University of Science and Medicine, Colton, CA 92324, USA
8
Department of Molecular and Internal Medicine, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima 734-8551, Japan
9
Medical Service, G.V. (Sonny) Montgomery VA Medical Center, Department of Pharmacology and Toxicology, Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Curr. Issues Mol. Biol. 2022, 44(1), 128-138; https://doi.org/10.3390/cimb44010010
Submission received: 22 November 2021 / Revised: 18 December 2021 / Accepted: 21 December 2021 / Published: 28 December 2021
(This article belongs to the Special Issue Molecules at Play in Cancer)
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Abstract

Primary aldosteronism is most often caused by aldosterone-producing adenoma (APA) and bi-lateral adrenal hyperplasia. Most APAs are caused by somatic mutations of various ion channels and pumps, the most common being the inward-rectifying potassium channel KCNJ5. Germ line mutations of KCNJ5 cause familial hyperaldosteronism type 3 (FH3), which is associated with severe hyperaldosteronism and hypertension. We present an unusual case of FH3 in a young woman, first diagnosed with primary aldosteronism at the age of 6 years, with bilateral adrenal hyperplasia, who underwent unilateral adrenalectomy (left adrenal) to alleviate hyperaldosteronism. However, her hyperaldosteronism persisted. At the age of 26 years, tomography of the remaining adrenal revealed two different adrenal tumors, one of which grew substantially in 4 months; therefore, the adrenal gland was removed. A comprehensive histological, immunohistochemical, and molecular evaluation of various sections of the adrenal gland and in situ visualization of aldosterone, using matrix-assisted laser desorption/ionization imaging mass spectrometry, was performed. Aldosterone synthase (CYP11B2) immunoreactivity was observed in the tumors and adrenal gland. The larger tumor also harbored a somatic β-catenin activating mutation. Aldosterone visualized in situ was only found in the subcapsular regions of the adrenal and not in the tumors. Collectively, this case of FH3 presented unusual tumor development and histological/molecular findings.
Keywords: familial hyperaldosteronism type 3; KCNJ5; adrenal tumor; β-catenin; MALDI-IMS; CYP11B2 familial hyperaldosteronism type 3; KCNJ5; adrenal tumor; β-catenin; MALDI-IMS; CYP11B2

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MDPI and ACS Style

Takizawa, N.; Tanaka, S.; Nishimoto, K.; Sugiura, Y.; Suematsu, M.; Ohe, C.; Ohsugi, H.; Mizuno, Y.; Mukai, K.; Seki, T.; et al. Familial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study. Curr. Issues Mol. Biol. 2022, 44, 128-138. https://doi.org/10.3390/cimb44010010

AMA Style

Takizawa N, Tanaka S, Nishimoto K, Sugiura Y, Suematsu M, Ohe C, Ohsugi H, Mizuno Y, Mukai K, Seki T, et al. Familial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study. Current Issues in Molecular Biology. 2022; 44(1):128-138. https://doi.org/10.3390/cimb44010010

Chicago/Turabian Style

Takizawa, Nae, Susumu Tanaka, Koshiro Nishimoto, Yuki Sugiura, Makoto Suematsu, Chisato Ohe, Haruyuki Ohsugi, Yosuke Mizuno, Kuniaki Mukai, Tsugio Seki, and et al. 2022. "Familial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study" Current Issues in Molecular Biology 44, no. 1: 128-138. https://doi.org/10.3390/cimb44010010

APA Style

Takizawa, N., Tanaka, S., Nishimoto, K., Sugiura, Y., Suematsu, M., Ohe, C., Ohsugi, H., Mizuno, Y., Mukai, K., Seki, T., Oki, K., Gomez-Sanchez, C. E., & Matsuda, T. (2022). Familial Hyperaldosteronism Type 3 with a Rapidly Growing Adrenal Tumor: An In Situ Aldosterone Imaging Study. Current Issues in Molecular Biology, 44(1), 128-138. https://doi.org/10.3390/cimb44010010

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