Zhang JM, Zheng CW, Li XW, Fang ZY, Yu MX, Shen HY, Ji X. Typical Zollinger-Ellison syndrome-atypical location of gastrinoma and absence of hypergastrinemia: A case report and review of literature. World J Clin Cases 2023; 11(26): 6223-6230 [PMID: 37731553 DOI: 10.12998/wjcc.v11.i26.6223]
Corresponding Author of This Article
Xia Ji, MD, Chief Physician, Department of Gastroenterology, The Second Affiliated Hospital of Jiaxing University, No. 1518 North Round-City Road, Nanhu District, Jiaxing 314001, Zhejiang Province, China. jixia1234@163.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
Jin-Ming Zhang, Chu-Wei Zheng, Zhi-Yun Fang, Hai-Yan Shen, Xia Ji, Department of Gastroenterology, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314001, Zhejiang Province, China
Xiao-Wen Li, Department of Pathology, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314001, Zhejiang Province, China
Mu-Xin Yu, College of Medicine, Jiaxing University, Jiaxing 314001, Zhejiang Province, China
Author contributions: Zhang JM was responsible for writing the paper; Zheng CW was responsible for patient treatment and study design; Fang ZY, Li XW and Yu MX were responsible for collecting and analyzing data; Shen HY and Ji X were responsible for patient follow-up.
Supported byNational Natural Science Foundation of China, No. 82100612; and Zhejiang Medical Health Science and Technology Plan Project, No. 2021KY1116.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and all accompanying images.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
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: Xia Ji, MD, Chief Physician, Department of Gastroenterology, The Second Affiliated Hospital of Jiaxing University, No. 1518 North Round-City Road, Nanhu District, Jiaxing 314001, Zhejiang Province, China. jixia1234@163.com
Received: May 18, 2023 Peer-review started: May 18, 2023 First decision: June 13, 2023 Revised: June 25, 2023 Accepted: August 11, 2023 Article in press: August 11, 2023 Published online: September 16, 2023
Abstract
BACKGROUND
Zollinger–Ellison syndrome (ZES) results from hypersecretion of gastrin from pancreatic or duodenal neuroendocrine tumors, commonly referred to as gastrinomas. The high levels of gastrin lead to a typical presentation involving watery diarrhea and multiple ulcers in the duodenum. Here, we have presented the rare case of a patient with ZES and absence of hypergastrinemia as well as an atypical location of gastrinoma.
CASE SUMMARY
A 72-year-old woman presented with the typical clinical manifestations of ZES, including upper abdominal pain, significant watery diarrhea, and acidic liquid vomitus. Surprisingly, however, she did not have an increased level of serum gastrin. In addition, there was no evidence of gastrinoma or any other ulcerogenic tumor. Esophagogastroduodenoscopy was conducted to examine the upper digestive tract. Revised diagnoses were considered, and an individualized treatment plan was developed. The patient responded to antacid medication while experiencing intermittent, recurring bouts of ZES. 18F-AlF-NOTA-octreotide positron emission tomography (18F-OC PET)/computed tomography (CT) helped locate the tumor. Postoperative pathology and immunohistochemistry results suggested that the tumor was a gastrinoma located at an unconventional site.
CONCLUSION
This present case study demonstrates the possibility of ZES-like manifestation in patients with absence of hypergastrinemia. 18F-OC PET/CT is a relatively new imaging technique that can be applied for diagnosing even tiny gastrinomas that are atypical in terms of location.
Core Tip: We recommend gastrinoma as one possible diagnosis for patients presenting Zollinger–Ellison syndrome-like clinical features, but without any typical tumor location or with normal serum gastrin levels. 18F-AlF-NOTA-octreotide positron emission tomography/computed tomography is an advanced imaging technique that can be used to locate and diagnose gastrinoma in such patients.
