title: Ectopic ACTH syndrome: our experience with 25 cases.
abstract: OBJECTIVE
  Ectopic ACTH syndrome (EAS) occurs in about 5-10% of all patients with ACTH-dependent hypercortisolism with most of them caused by intrathoracic neoplasms. It may be associated with overt malignancies or with occult and indolent tumors. We assessed the accuracy of dynamic tests, inferior petrosal sinus sampling (IPSS) using desmopressin, and imaging in the work-up diagnosis of EAS.
  
  
  METHODS
  Tumor markers, imaging, and outcome data from 25 patients (13F/12M) aged 18-72 years. High dexamethasone suppression test (HDDST), desmopressin test, GHRP-6 test, corticotropin-releasing hormone (CRH) test, IPSS, computed tomography (CT), magnetic resonance imaging (MRI), and (111)In-pentetreotide scintigraphy were revised.
  
  
  RESULTS
  In 5 out of 20 patients HDDST was positive. In 13 patients who underwent desmopressin test, ACTH- and cortisol-positive responses were seen in six and five patients respectively. GHRP-6 test was positive in two out of three cases. Two patients underwent CRH test with negative response. In the seven patients submitted to IPSS using desmopressin in six of them, none had ACTH gradients. CT was positive in 15 out of 21 patients and MRI in 8 out of 17 cases. (111)In-pentetreotide scintigraphy was positive in three out of five patients. Fourteen patients had intrathoracic tumors, five had pheochromocytomas, three had pancreatic tumors, one had a glomic tumor, and had three occult tumors. Six out of 11 patients with metastasis died and 3 others without metastasis died.
  
  
  CONCLUSIONS
  IPSS with desmopressin was helpful for differential diagnosis. Patients initially harboring occult carcinoids may also exhibit severe hypercortisolism and those harboring tymic carcinoids had poor prognoses when compared with bronchial carcinoids and pheocromocytomas.
authors.affiliation: Division of Endocrinology and Metabolism, Hospital das Clínicas, University of Sao Paulo Medical School, Avenida Dr Eneas de Carvalho Aguiar, 155, 8th floor, Cerqueira Cesar, 05403-060 Sao Paulo, Brazil. salga@uol.com.br
authors.forename: Luiz Roberto
authors.initials: LR
authors.lastname: Salgado
authors.forename: Maria Candida B Villares
authors.initials: MC
authors.lastname: Fragoso
authors.forename: Mirta
authors.initials: M
authors.lastname: Knoepfelmacher
authors.forename: Marcio Carlos
authors.initials: MC
authors.lastname: Machado
authors.forename: Sorahia
authors.initials: S
authors.lastname: Domenice
authors.forename: Maria Adelaide Albergaria
authors.initials: MA
authors.lastname: Pereira
authors.forename: Berenice Bilharinho
authors.initials: BB
authors.lastname: de Mendonça
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis* / Q000628:therapy; D049913:ACTH-Secreting Pituitary Adenoma / Q000175:diagnosis; D000293:Adolescent; D000328:Adult; D000368:Aged; D003346:Corticotropin-Releasing Hormone; D003894:Deamino Arginine Vasopressin; D003907:Dexamethasone; D005260:Female; D006801:Humans; D007008:Hypokalemia / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009842:Oligopeptides; D010901:Pituitary Function Tests; D016896:Treatment Outcome
chemical_list: D009842:Oligopeptides; C041048:growth hormone releasing hexapeptide; D003907:Dexamethasone; D003346:Corticotropin-Releasing Hormone; D003894:Deamino Arginine Vasopressin