title: An extra-adrenal abdominal pheochromocytoma causing ectopic ACTH syndrome.
abstract: We report a 55-year-old woman with ectopic adrenocorticotropin (ACTH) secretion caused by extra-adrenal pheochromocytoma. The patient presented with a 6-month history of hypertension and diabetes mellitus. Her serum and urinary cortisol levels were extremely high and dexamethasone failed to suppress the cortisol secretion. Her plasma ACTH levels were also elevated (>300 pg/mL) and irresponsive to corticotropin-releasing hormone (CRH) or metyrapone administration. Gel filtration analysis of the patient's plasma detected the existence of large molecular weight ACTH being eluted with a major peak of authentic 1-39 ACTH. Abdominal computed tomographic scan and magnetic resonance imaging revealed a 5-cm paraganglioma located underneath the left kidney, in which (123)I-MIBG tracer specifically accumulated. Bilateral adrenal glands were diffusely enlarged. After surgical removal of the paraganglioma, the patient's clinical symptoms improved and biochemistry normalized including plasma ACTH, urinary free cortisol, and urinary catecholamines. Subsequent histologic evaluation of the transected paranglioma tissue revealed ACTH, synaptin, and chromogranin-A histologically immunostaining. Culture of primary cells collected from the resected paraganglioma demonstrated of in vitro production of ACTH, noradrenaline, and adrenaline. This is the first report of ectopic ACTH syndrome induced by an extra-adrenal abdominal paraganglioma.
authors.affiliation: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan. fumiotsu@md.okayama-u.ac.jp
authors.forename: Fumio
authors.initials: F
authors.lastname: Otsuka
authors.forename: Tomoko
authors.initials: T
authors.lastname: Miyoshi
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Murakami
authors.forename: Kenichi
authors.initials: K
authors.lastname: Inagaki
authors.forename: Masaya
authors.initials: M
authors.lastname: Takeda
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Ujike
authors.forename: Toshio
authors.initials: T
authors.lastname: Ogura
authors.forename: Masako
authors.initials: M
authors.lastname: Omori
authors.forename: Hiroyoshi
authors.initials: H
authors.lastname: Doihara
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Kozo
authors.initials: K
authors.lastname: Hashimoto
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis / Q000209:etiology*; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis; D000324:Adrenocorticotropic Hormone / Q000097:blood; D002395:Catecholamines / Q000652:urine; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000652:urine; D008875:Middle Aged; D010235:Paraganglioma; D010673:Pheochromocytoma / Q000150:complications* / Q000175:diagnosis; D011860:Radiography, Abdominal; D014057:Tomography, X-Ray Computed
chemical_list: D002395:Catecholamines; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone