Ectopic ACTH syndrome: our experience with 25 cases.
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.
Salgado
Luiz Roberto
LR
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
Fragoso
Maria Candida B Villares
MC
Knoepfelmacher
Mirta
M
Machado
Marcio Carlos
MC
Domenice
Sorahia
S
Pereira
Maria Adelaide Albergaria
MA
de Mendonça
Berenice Bilharinho
BB
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
D009842:Oligopeptides; C041048:growth hormone releasing hexapeptide; D003907:Dexamethasone; D003346:Corticotropin-Releasing Hormone; D003894:Deamino Arginine Vasopressin