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An aberrant ACTH-producing ectopic pituitary adenoma in the sphenoid sinus.
Endocr J. 2004 Feb; 51(1):97-103.EJ

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 microg/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.

Authors+Show Affiliations

Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

15004415

Citation

Suzuki, Jiro, et al. "An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus." Endocrine Journal, vol. 51, no. 1, 2004, pp. 97-103.
Suzuki J, Otsuka F, Ogura T, et al. An aberrant ACTH-producing ectopic pituitary adenoma in the sphenoid sinus. Endocr J. 2004;51(1):97-103.
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.
Suzuki J, et al. An Aberrant ACTH-producing Ectopic Pituitary Adenoma in the Sphenoid Sinus. Endocr J. 2004;51(1):97-103. PubMed PMID: 15004415.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - An aberrant ACTH-producing ectopic pituitary adenoma in the sphenoid sinus. AU - Suzuki,Jiro, AU - Otsuka,Fumio, AU - Ogura,Toshio, AU - Kishida,Masayuki, AU - Takeda,Masaya, AU - Tamiya,Takashi, AU - Nishioka,Tatsuya, AU - Tanaka,Yasushi, AU - Hashimoto,Kozo, AU - Makino,Hirofumi, PY - 2004/3/9/pubmed PY - 2004/11/13/medline PY - 2004/3/9/entrez SP - 97 EP - 103 JF - Endocrine journal JO - Endocr J VL - 51 IS - 1 N2 - 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 microg/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. SN - 0918-8959 UR - https://www.unboundmedicine.com/medline/citation/15004415/An_aberrant_ACTH_producing_ectopic_pituitary_adenoma_in_the_sphenoid_sinus_ L2 - https://joi.jlc.jst.go.jp/JST.JSTAGE/endocrj/51.97?from=PubMed DB - PRIME DP - Unbound Medicine ER -