The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up.
BACKGROUND
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.


METHODS
This was a retrospective case-record study.


METHODS
The setting for this study was a tertiary referral hospital center.


METHODS
Forty patients with EAS were studied.


METHODS
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.
Isidori
Andrea M
AM
Department of Endocrinology, St. Bartholomew's Hospital, London EC1A 7BE, United Kingdom.
Kaltsas
Gregory A
GA
Pozza
Carlotta
C
Frajese
Vanni
V
Newell-Price
John
J
Reznek
Rodney H
RH
Jenkins
Paul J
PJ
Monson
John P
JP
Grossman
Ashley B
AB
Besser
G Michael
GM
D000182:ACTH Syndrome, Ectopic / Q000097:blood / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001984:Bronchial Neoplasms / Q000097:blood / Q000378:metabolism; D002276:Carcinoid Tumor / Q000097:blood / Q000378:metabolism; D003480:Cushing Syndrome / Q000175:diagnosis / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000097:blood / Q000378:metabolism*; D018358:Neuroendocrine Tumors / Q000097:blood / Q000378:metabolism; D012189:Retrospective Studies; D016019:Survival Analysis
D000324:Adrenocorticotropic Hormone