title: Atypical Cushing's syndrome caused by ectopic ACTH secretion of an oesophageal adenocarcinoma.
abstract: We present an atypical case of Cushing's syndrome caused by ectopic adrenocorticotropic hormone (ACTH) secretion in a patient with a metastasised adenocarcinoma of the oesophagus. After chemotherapy and surgery the patient developed generalised oedema, hyperpigmentation and dysphagia. Laboratory tests showed hypokalaemia, normal urinary potassium, increased 24-hour urinary free cortisol excretion and serum ACTH within the normal reference range. The diagnosis of ACTH-dependent Cushing's syndrome was made, most probably caused by ectopic production of ACTH. In addition to combined chemotherapy, treatment with ketoconazole sufficiently reduced urinary cortisol excretion and relieved the symptoms.
authors.affiliation: Department of Clinical Oncology, Leiden University Medical Centre, Leiden, the Netherlands. j.m.baas@lumc.nl
authors.forename: J M
authors.initials: JM
authors.lastname: Baas
authors.forename: E
authors.initials: E
authors.lastname: Kapiteijn
authors.forename: A M
authors.initials: AM
authors.lastname: Pereira
authors.forename: J W R
authors.initials: JW
authors.lastname: Nortier
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000150:complications / Q000209:etiology*; D000230:Adenocarcinoma / Q000378:metabolism*; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D003480:Cushing Syndrome / Q000188:drug therapy / Q000209:etiology*; D004938:Esophageal Neoplasms / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged
chemical_list: D000324:Adrenocorticotropic Hormone