Adrenocorticotropin-independent unilateral adrenocortical hyperplasia with Cushing's syndrome: Immunohistochemical studies of steroidogenic enzymes, ultrastructural examination and a review of the literature

Toshinari Takamura, Yukihiro Nagai, Masayuki Taniguchi, Haruhisa Yamashita, Saburo Nakamura, Takayuki Ikeda, Ken Ichi Kobayashi, Takashi Suzuki, Hironobu Sasano

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)

Abstract

A 60-year-old woman presented with a history of palpitations, headaches and severe hypertension, which was resistant to hypotensive agents. She had a 2-year history of obesity and a moon face. Her plasma adrenocorticotropic hormone level was below the limits of detection and did not respond to corticotropin-releasing hormone. Urinary-free cortisol was elevated and the circadian rhythm of serum cortisol level had completely disappeared. Imaging analysis demonstrated a unilaterally functioning mass in the left adrenal gland. Serum cortisol level in the left adrenal vein was elevated. The resected adrenal mass measured 4 × 3.5 × 2.5 cm, and ranged from yellow to tan in color. The adrenal cortex adjacent to the nodule did not demonstrate cortical atrophy. The mass was well circumscribed but not encapsulated, and consisted of multiple cortical nodules. These nodules were composed predominantly of clear cortical cells, and partly of compact cortical cells. Immunoreactivity of steroidogenic enzymes including cholesterol side-chain-cleavage P450, 3β-hydroxysteroid dehydrogenase, 21-hydroxylase cytechrome P450, 11β-hydroxylase cytochrome P450 and 17α-hydroxylase cytochrome P450 was marked in cortical nodules, but minimal in non-nodular cortex. Ultrastructural examination of nodular cortical cells also demonstrated well-developed mitochondria and smooth endoplasmic reticulum, consistent with elevated steroidogenesis in these cells.

Original languageEnglish
Pages (from-to)118-122
Number of pages5
JournalPathology international
Volume51
Issue number2
DOIs
Publication statusPublished - 2001

Keywords

  • Adrenal hyperplasia
  • Cushing's syndrome
  • Immunohistochemistry
  • Steroidogenic enzyme

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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