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

• SUZUKI Jiro

  Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

• OTSUKA Fumio

  Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

• OGURA Toshio

  Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

• KISHIDA Masayuki

  Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

• TAKEDA Masaya

  Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

• TAMIYA Takashi

  Department of Nurosurgery, Okayama University Graduate School of Medicine and Dentistry

• NISHIOKA Tatsuya

  Cinical Laboratories, Kochi Medical School

• TANAKA Yasushi

  Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School

• HASHIMOTO Kozo

  Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School

• MAKINO Hirofumi

  Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry

抄録

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.<br>

収録刊行物

• Endocrine Journal

  Endocrine Journal 51 (1), 97-103, 2004

  一般社団法人 日本内分泌学会

キーワード

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

詳細情報

• CRID: 1390282681277511296
• NII論文ID: 130004443106
• NII論文ID: 80016446144
• NII書誌ID: AA10901436
• DOI: 10.1507/endocrj.51.97
• ISSN: 13484540
• ISSN: 09188959
• PubMed: 15004415
• Web Site: http://www.jstage.jst.go.jp/article/endocrj/51/1/51_1_97/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2004194463
• 本文言語コード: en
• データソース種別:
  • JaLC
  • Crossref
  • PubMed
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可