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lüll The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up Isidori AM; Kaltsas GA; Pozza C; Frajese V; Newell-Price J; Reznek RH; Jenkins PJ; Monson JP; Grossman AB; Besser GMJ Clin Endocrinol Metab 2006[Feb]; 91 (2): 371-7CONTEXT: There are few large series of patients with ectopic, nonpituitary, corticotropin (ACTH) secretion (EAS). OBJECTIVE: The objective of this study was to analyze the clinical, biochemical, and radiological features, management, and treatment outcome of patients with EAS. DESIGN: This was a retrospective case-record study. SETTING: The setting for this study was a tertiary referral hospital center. PATIENTS: Forty patients with EAS were studied. MAIN OUTCOME MEASURES: Clinical, biochemical, and radiological features and response to therapy and survival were measured. RESULTS: The median follow-up was 5 yr (range, 2-30 yr). None of the dynamic tests achieved 100% accuracy, but bilateral inferior petrosal sinus sampling showed an absent central gradient in all but one case (one of 12). Imaging correctly identified the lesion at first investigation in 65% of cases. Bronchial carcinoid tumors were the most common cause of EAS (n = 12; 30%), followed by other neuroendocrine tumors (n = 13, 32.5%). In 12.5% of patients, the source of EAS was never found. Octreotide scintigraphy and whole-body venous sampling were of limited value. Surgical attempt at curative resection was successful in 83% (10 of 12) of patients with bronchial carcinoid tumors; others responded generally well to adrenolytic therapy or bilateral adrenalectomy. Tumor histology and the presence of distant metastases were the main predictors of overall survival (P < 0.05). CONCLUSIONS: A variety of tests and imaging studies are necessary for the correct diagnosis of the EAS, but even then, up to 20% of cases present a covert or occult EAS syndrome. These cases require a prolonged follow-up, review, and repetition of diagnostic tests and scans.|ACTH Syndrome, Ectopic/blood/*diagnosis/pathology/*therapy[MESH]|Adolescent[MESH]|Adrenocorticotropic Hormone/blood[MESH]|Adult[MESH]|Aged[MESH]|Aged, 80 and over[MESH]|Bronchial Neoplasms/blood/metabolism[MESH]|Carcinoid Tumor/blood/metabolism[MESH]|Cushing Syndrome/diagnosis/pathology[MESH]|Diagnosis, Differential[MESH]|Female[MESH]|Follow-Up Studies[MESH]|Humans[MESH]|Male[MESH]|Middle Aged[MESH]|Neoplasms/blood/*metabolism[MESH]|Neuroendocrine Tumors/blood/metabolism[MESH]|Retrospective Studies[MESH]|Survival Analysis[MESH] |