Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2018; 6(14): 862-868
Published online Nov 26, 2018. doi: 10.12998/wjcc.v6.i14.862
Octreotide reverses shock due to vasoactive intestinal peptide-secreting adrenal pheochromocytoma: A case report and review of literature
Xiao Hu, Wei Cao, Min Zhao
Xiao Hu, Min Zhao, Department of Emergency Medicine, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
Wei Cao, Department of Pathology, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
Author contributions: Zhao M and Hu X designed the report; Hu X collected the patient’s clinical data; Cao W performed the pathological and immunohistopathological studies; Zhao M and Hu X analyzed the data and wrote the paper.
Supported by National Clinical Specialty Construction Project, No. 2012649.
Informed consent statement: Consent was obtained from relatives of the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Min Zhao, MD, PhD, Professor, Chief, Department of Emergency Medicine, Shengjing Hospital of China Medical University, No. 36, Sanhao Street, Shenyang 110004, Liaoning Province, China. zhaom@sj-hospital.org
Telephone: +86-24-9661564131 Fax: +86-24-9661564131
Received: September 5, 2018
Peer-review started: September 5, 2018
First decision: October 4, 2018
Revised: October 15, 2018
Accepted: October 17, 2018
Article in press: October 16, 2018
Published online: November 26, 2018
Processing time: 83 Days and 1.2 Hours
Abstract

Vasoactive intestinal peptide-producing tumors (VIPoma) usually originate in the pancreas and are characterized by diarrhea, hypokalemia, and achlorhydria (WDHA syndrome). In adults, nonpancreatic VIPoma is very rare. Herein, we report an unusual case of VIP-producing pheochromocytoma marked by persistent shock, flushing, and watery diarrhea and high sensitivity to octreotide. A 53-year-old woman was hospitalized for sudden-onset hypertension with convulsions, which then rapidly evolved to persistent shock, flushing, and watery diarrhea. Abdominal computed tomography indicated a left adrenal mass, accompanied by bleeding; and marked elevations of both plasma catecholamine and VIP concentrations were documented via laboratory testing. Surprisingly, all clinical symptoms responded swiftly to octreotide treatment. Once surgically treated, hormonal levels normalized in this patient, and the clinical symptoms dissipated. Postoperative pathological and immunohistopathological studies confirmed a VIP-secreting pheochromocytoma with strong, diffuse positivity for somatostatin receptor type 2. During a 6-mo follow-up period, she seemed in good health and was symptom-free.

Keywords: Pheochromocytoma; Vasoactive intestinal peptide; Octreotide; Shock; Flushing; Diarrhea; Case report

Core tip: Vasoactive intestinal peptide-producing tumors (VIPoma) usually originate in the pancreas. VIP-secreting pheochromocytoma is very rare and most of the related cases reported are characterized by diarrhea, hypokalemia, and gastric acid deficiency (WDHA syndrome). To our knowledge, this is the first reported instance of VIP-secreting pheochromocytoma marked by persistent shock flushing and diarrhea and high sensitivity to octreotide. This case helps to improve the understanding of the pathogenesis, biology, and behavior of VIPoma and pheochromocytoma.