An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus

Jiro Suzuki, Fumio Otsuka, Toshio Ogura, Masayuki Kishida, Masaya Takeda, Takashi Tamiya, Tatsuya Nishioka, Yasushi Tanaka, Kozo Hashimoto, Hirofumi Makino

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

A 32-year-old woman with an ectopic adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma (EAPA) is presented. She had rapidly gained weight and suffered recurrent facial acne for a few years but lacked the typical Cushingoid features. Endocrine examinations revealed that her plasma ACTH was markedly high (196 to 280 pg/ml) without showing normal circadian rhythm and failed to respond to corticotropin-releasing hormone stimulation. Her cortisol levels ranged from 22 to 30 μ/d1 throughout observation but low doses (1 and 2 mg) of dexamethasone failed to suppress either ACTH or cortisol level. Magnetic resonance imaging study revealed a 3-cm mass occupying the sphenoidal sinus with partial enhancement by gadolinium, which was separated from the normal pituitary in the sella region. The tumor resected by transsphenoidal surgery was histologically diagnosed as an ACTH-producing pituitary adenoma. After surgery her weight gain and acne remitted in accordance with decreases in plasma ACTH. Analysis of patient plasma by gel filtration method revealed the existence of big ACTH molecules eluted with a peak of authentic 1-39 ACTH, suggesting that this biologically less-active ACTH might be the reason why overt features of Cushing's syndrome failed to develop in this case. Although EAPA is clinically rare in parasellar disorders, the presence of ectopic pituitary adenoma should be considered in such cases showing ACTH hypersecretion without typical Cushingoid features.

Original languageEnglish
Pages (from-to)97-103
Number of pages7
JournalEndocrine journal
Volume51
Issue number1
DOIs
Publication statusPublished - Feb 2004

Keywords

  • Adrenocorticotropin (ACTH)
  • Cushing's disease
  • Cushing's syndrome
  • Ectopic ACTH syndrome
  • Gel filtration analysis
  • Pituitary adenoma

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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    Suzuki, J., Otsuka, F., Ogura, T., Kishida, M., Takeda, M., Tamiya, T., Nishioka, T., Tanaka, Y., Hashimoto, K., & Makino, H. (2004). An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus. Endocrine journal, 51(1), 97-103. https://doi.org/10.1507/endocrj.51.97