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

doi:10.1507/endocrj.51.97

ORIGINALS

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

Author information

Jiro SUZUKI
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry
Fumio OTSUKA
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry
Toshio OGURA
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry
Masayuki KISHIDA
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry
Masaya TAKEDA
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry
Takashi TAMIYA
Department of Nurosurgery, Okayama University Graduate School of Medicine and Dentistry
Tatsuya NISHIOKA
Cinical Laboratories, Kochi Medical School
Yasushi TANAKA
Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School
Kozo HASHIMOTO
Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School
Hirofumi MAKINO
Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

Keywords: Cushing's syndrome, Cushing's disease, Pituitary adenoma, Adrenocorticotropin (ACTH), Ectopic ACTH syndrome, Gel filtration analysis

JOURNAL FREE ACCESS

2004 Volume 51 Issue 1 Pages 97-103

DOI https://doi.org/10.1507/endocrj.51.97

Details

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 μg/dl 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.

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