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Case Reports in Endocrinology
Volume 2018, Article ID 6389374, 5 pages
Case Report

A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection

1Vanderbilt University Medical Center, Division of Endocrinology, Department of Medicine, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USA
2Johns Hopkins University School of Medicine, Division of Endocrinology, Diabetes and Metabolism, 5501 Hopkins Bayview Circle, Baltimore, MD 21224, USA
3Vanderbilt University Medical Center, Department of Surgery, Vanderbilt University, 1161 21st Avenue South, Nashville, TN 37232, USA
4Vanderbilt University School of Medicine, 2215 Garland Ave, Nashville, TN 37232, USA
5Vanderbilt University Medical Center, Department of Pathology, Microbiology and Immunology, 1161 21st Avenue South, Nashville, TN 37232, USA

Correspondence should be addressed to Shichun Bao; ude.tlibrednav@oab.nuhcihs

Received 6 September 2017; Accepted 20 December 2017; Published 4 February 2018

Academic Editor: Eli Hershkovitz

Copyright © 2018 Leslee N. Matheny et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


We present a case of a 52-year-old male who developed Cushing’s Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Work-up revealed ACTH-dependent hypercortisolemia, which did not suppress appropriately after high-dose dexamethasone. Subsequent imaging of the chest, abdomen, and pelvis did not reveal other possible ectopic sources of ACTH secretion besides the ENB. His hospital course was complicated by severe hypokalemia and hyperglycemia before successful surgical resection of the tumor, the biopsy of which showed ENB. Postoperatively, his ACTH level dropped below the limit of detection. In the ensuing 4 months, he underwent adjuvant chemoradiation with carboplatin and docetaxel with good response and resolution of hypokalemia and hyperglycemia, with no sign of recurrence as of 30 months postoperatively. His endogenous cortisol production is rising but has not completely recovered.