title: Cushing's syndrome due to ectopic ACTH production by (neuroendocrine) prostate carcinoma.
abstract: Ectopic adrenocorticotropin (ACTH) secretion accounts for less than 10% of all causes of endogenous Cushing's syndrome (CS) and is usually associated with neuroendocrine tumors and small cell carcinoma of the lung. We report the case of a 62-year-old man with CS due to ectopic ACTH production by small cell carcinoma of the prostate. He presented with severe hypercortisolism and associated symptoms. Plasma neuron specific enolase (NSE) was grossly elevated. Despite performing a laparoscopic bilateral adrenalectomy, the patient died as a result of sepsis with multi-organ failure. Post-mortem immunohistochemical staining of prostate tumor tissue showed ACTH expression. ACTH staining was also performed in four additional patients with small cell carcinoma of the urinary tract without CS. None of these additional cases showed a positive staining for ACTH. Although a rare cause of ectopic ACTH production, neuroendocrine prostate carcinoma should be considered in male patients with Cushing's syndrome, in particular in those with an occult source of ACTH overproduction.
authors.affiliation: Department of Internal Medicine, Endocrine Section, Rotterdam, The Netherlands. r.alwani@erasmusmc.nl
authors.forename: R A
authors.initials: RA
authors.lastname: Alwani
authors.forename: S J C M M
authors.initials: SJ
authors.lastname: Neggers
authors.forename: M
authors.initials: M
authors.lastname: van der Klift
authors.forename: M G A
authors.initials: MG
authors.lastname: Baggen
authors.forename: G J L H
authors.initials: GJ
authors.lastname: van Leenders
authors.forename: M O
authors.initials: MO
authors.lastname: van Aken
authors.forename: A J
authors.initials: AJ
authors.lastname: van der Lely
authors.forename: W W
authors.initials: WW
authors.lastname: de Herder
authors.forename: R A
authors.initials: RA
authors.lastname: Feelders
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D003480:Cushing Syndrome / Q000209:etiology* / Q000378:metabolism* / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008875:Middle Aged; D011471:Prostatic Neoplasms / Q000150:complications* / Q000378:metabolism*; D055752:Small Cell Lung Carcinoma / Q000378:metabolism*
chemical_list: D000324:Adrenocorticotropic Hormone