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©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
Ectopic Cushing's syndrome in a patient with metastatic Merkel cell carcinoma: A case report
Avraham Ishay, Elia Touma, Olga Vornicova, Roni Dodiuk-Gad, Tal Goldman, Naiel Bisharat
Avraham Ishay, Endocrine and Diabetes Unit, Emek Medical Center, Afula 18134, Israel
Avraham Ishay, Roni Dodiuk-Gad, Naiel Bisharat, Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 3200003, Israel
Elia Touma, Department of Internal Medicine D, Emek Medical Center, Afula 18134, Israel
Olga Vornicova, Department of Oncology, Emek Medical Center, Afula 18134, Israel
Roni Dodiuk-Gad, Department of Dermatology, Emek Medical Center, Afula 18134, Israel
Tal Goldman, Department of Pathology, Emek Medical Center, Afula 18134, Israel
Naiel Bisharat, Department of Medicine D, Emek Medical Center, Afula 18134, Israel
Author contributions: Ishay A and Touma E were the patient's physicians, wrote the manuscript, and reviewed the literature; Vornicova O, Dodiuk Gad, and Bisharat N were involved in the patient treatment and contributed to manuscript drafting; Goldman T was the pathologist and prepared and interpreted the pathology images.
Informed consent statement: Not applicable.
Conflict-of-interest statement: All the authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
https://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Avraham Ishay, MD, Chief Physician, Senior Lecturer, Endocrine and Diabetes Unit, Emek Medical Center, Rabin Ave 21, Afula 18134, Israel. ishay_av@clalit.co.il
Received: January 21, 2022
Peer-review started: January 21, 2022
First decision: March 23, 2022
Revised: April 3, 2022
Accepted: June 13, 2022
Article in press: June 13, 2022
Published online: August 6, 2022
BACKGROUND
Ectopic Cushing syndrome (ECS) is a rare condition commonly associated with neuroendocrine tumors (NET), mainly bronchial carcinoids. The association of paraneoplastic syndrome with Merkle cell carcinoma (MCC) is limited to individual case reports.
CASE SUMMARY
In this article we report an unusual and striking presentation of ECS in a patient with known metastatic MCC. An elderly patient presented with new onset severe hypertension, hyperglycemia and hypokalemia, muscle wasting, and peripheral edema. A diagnosis of adrenocorticotropic hormone dependent, non-pituitary, Cushing syndrome was established. Medical therapy inhibiting adrenal function was promptly started but unfortunately the patient survived only a few days after diagnosis.
CONCLUSION
The occurrence of an aggressive form of ECS in patients with NET should be recognized as an ominous event. To our knowledge, the association of this complication in a patient with MCC had not been reported.
Core Tip: Merkel cell carcinoma (MCC) is an uncommon but highly aggressive skin cancer with neuroendocrine features. Its incidence and mortality are increasing. We describe an elderly patient with a 2-year history of metastatic MCC, with no apparent cutaneous lesion at diagnosis, who presented with uncontrolled hypertension, diabetes mellitus, and hypokalemia. A diagnosis of ectopic Cushing syndrome was established. The occurrence of ectopic Cushing syndrome in patients with neuroendocrine tumor is a major cause of poor prognosis. To our knowledge, this is the first reported case of ectopic Cushing syndrome linked to the rapid progression of a metastatic MCC.
