A Case of Cortisol Producing Adrenal Adenoma without Phenotype of Cushing's Syndrome due to Impaired 11β-Hydroxysteroid Dehydrogenase 1 Activity

Hiroshi ARAI; Nozomi KOBAYASHI; Yuko NAKATSURU; Hiroaki MASUZAKI; Takuo NAMBU; Kazuhiko TAKAYA; Yusuke YAMANAKA; Eri KONDO; Go YAMADA; Toshihito FUJII; Masako MIURA; Yasato KOMATSU; Naotetsu KANAMOTO; Hiroyuki ARIYASU; Kenji MORIYAMA; Akihiro YASODA; Kazuwa NAKAO

doi:10.1507/endocrj.K08E-008

ORIGINALS

A Case of Cortisol Producing Adrenal Adenoma without Phenotype of Cushing's Syndrome due to Impaired 11β-Hydroxysteroid Dehydrogenase 1 Activity

Hiroshi ARAI, Nozomi KOBAYASHI, Yuko NAKATSURU, Hiroaki MASUZAKI, Takuo NAMBU, Kazuhiko TAKAYA, Yusuke YAMANAKA, Eri KONDO, Go YAMADA, Toshihito FUJII, Masako MIURA, Yasato KOMATSU, Naotetsu KANAMOTO, Hiroyuki ARIYASU, Kenji MORIYAMA, Akihiro YASODA, Kazuwa NAKAO

Author information

Hiroshi ARAI
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Nozomi KOBAYASHI
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Yuko NAKATSURU
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Hiroaki MASUZAKI
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Takuo NAMBU
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Kazuhiko TAKAYA
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Yusuke YAMANAKA
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Eri KONDO
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Go YAMADA
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Toshihito FUJII
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Masako MIURA
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Yasato KOMATSU
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Naotetsu KANAMOTO
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Hiroyuki ARIYASU
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Kenji MORIYAMA
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Akihiro YASODA
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine
Kazuwa NAKAO
Department of Endocrinology and Metabolism, Kyoto University Graduate School of Medicine

Keywords: 11β-Hydroxysteroid Dehydrogenase 1(11β-HSD 1), Cortisol, Adrenal adenoma, Cushing's syndrome

JOURNAL FREE ACCESS

2008 Volume 55 Issue 4 Pages 709-715

DOI https://doi.org/10.1507/endocrj.K08E-008

View "Advance Publication" version (May 21, 2008).

Details

Abstract

This report concerns a case of cortisol-producing adrenocortical adenoma without the phenotype of Cushing's syndrome. A left adrenal tumor was incidentally detected in this patient. A diagnosis of adrenal Cushing's syndrome was based on the results of endocrinological and radiological examinations, although she showed none of the physical signs of Cushing's syndrome, glucose intolerance, hypertension or dyslipidermia. After a successful laparoscopic left adrenalectomy, the pathological diagnosis was adrenocortical adenoma. Slow tapering of glucocorticoids was needed to prevent adrenal insufficiency after surgery, and the plasma ACTH level remained high even though the serum cortisol level had reached the upper limit of the normal range. Further examination showed a urinary THF + allo-THF/THE ratio of 0.63, which was lower than that of control (0.90 ± 0.13, mean ± SD). Serum cortisol/cortisone ratios after the cortisone acetate administration were also decreased, and the serum half-life of cortisol was shorter than the normal range which has been reported. These findings indicated a partial defect in 11β-hydroxysteroid dehydrogenase 1 (11β-HSD1) activity, which converts cortisone to cortisol. Our case suggests that a change in 11β-HSD1 activity results in inter-individual differences in glucocorticoid efficacy.

Corresponding author

Register with J-STAGE for free!