ECE2018 Poster Presentations: Adrenal and Neuroendocrine Tumours Endocrine tumours and neoplasia (34 abstracts)
Ectopic adrenocorticotropic hormone syndrome: clinical features, diagnosis, treatment and long-term observation. Impact of bronchial carcinoid tumors
Vadim Krylov 1 , Ekaterina Dobreva 2 , Sergey Kharnas 1 , Vladimir Parshin 1 , Leonid Ippolitov 1 , Nikolay Kuznetsov 2 & Georgy Polunin 1
1Sechenov University, Moscow, Russian Federation; 2Endocrinology Research Centre, Moscow, Russian Federation.
Objective: Ectopic adrenocorticotropic hormone (ACTH-ectopic) syndrome (EAS) is a rare cause of ACTH-dependent endogenous hypercortisolism. The objective of this study was to analyze the clinical, biochemical, and radiological features, management, and treatment outcome of patients with EAS.
Methods: It was a retrospective case-record study of 47 patients with EAS. Clinical, biochemical, and radiological features and response to therapy and survival rate were measured.
Results: The median follow-up was 7 yrs. (range, 1–13 yrs.). None of the dynamic tests achieved 100% accuracy. Imaging correctly identified the lesion at first investigation in 80.9% of cases. Bronchial carcinoid tumors were the most common cause of EAS (n=27; 57.5%), followed by other neuroendocrine tumors (n=11, 23.4%). In 19.1% (9) of patients, the source of EAS was never found. Octreotide scintigraphy and whole-body venous sampling were of limited diagnostic value. Surgical attempt at curative resection was successful in 81% (38 out of 47) of all patients; 9 (19.1%) responded generally well to bilateral adrenalectomy by vital indication. Tumor histology and the presence of distant metastases were the main predictors of overall survival (P<0.05).
Conclusions: Bronchial carcinoid tumors is the main cause of ACTH-ectopic syndrome. No single test was capable of finding the source of EAS correctly. Despite a variety of tests and imaging studies for the correct diagnosis of the EAS, up to 19% of cases present as occult EAS syndrome. These cases require a prolonged follow-up, review, and repetition of diagnostic tests and scans, but, in severe cases, bilateral adrenalectomy is performed.
Volume 56
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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