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

Endocrine Journal

Takashi TAMIYA³  Kozo HASHIMOTO⁴  Toshio OGURA¹  Masaya TAKEDA¹  Hirofumi MAKINO¹  Fumio OTSUKA¹  Jiro SUZUKI¹  Masayuki KISHIDA¹  Tatsuya NISHIOKA²  Yasushi TANAKA⁴ 

[1] Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry;Cinical Laboratories, Kochi Medical School;Department of Nurosurgery, Okayama University Graduate School of Medicine and Dentistry;Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School

关键词: Cushing's syndrome;    Cushing's disease;    Pituitary adenoma;    Adrenocorticotropin (ACTH);    Ectopic ACTH syndrome;    Gel filtration analysis;   

DOI  :  10.1507/endocrj.51.97

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(31)Cited-By(15)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.

【 授权许可】

Unknown