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Submitted on July 12, 2005
Accepted on November 9, 2005
Departments of Endocrinology (A.M.I., G.A.K., C.P., V.J., P.J.J., J.P.M., A.B.G., G.M.B.) and Academic Radiology (R.H.R.), St Bartholomew's Hospital, London EC1A 7BE, UK; and Department of Endocrinology (J.N.P.), Sheffield University School of Medicine, UK
* To whom correspondence should be addressed. E-mail: a.b.grossman{at}qmul.ac.uk.
Context: There are few large series of patients with ectopic, non-pituitary, corticotropin (ACTH) secretion (EAS).
Objective: To analyze the clinical, biochemical and radiological features, management and treatment outcome of patients with EAS.
Design: Retrospective case-record study.
Setting: Tertiary referral hospital center.
Patients: 40 patients with EAS.
Main Outcome Measures: Clinical, biochemical and radiological features, response to therapy and survival.
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 (1/12). 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 (NET) (n = 13, 32.5%). In 12.5% of patients the source of ectopic ACTH secretion was never found. Octreotide scintigraphy and whole-body venous sampling were of limited value. Surgical attempt at curative resection was successful in 83% (10/12) 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. Theses cases require a prolonged follow-up, review and repetition of diagnostic tests and scans.
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