Accepted Manuscript. Classical features of Zollinger-Ellison syndrome, in images. Ali Alshati, MD, Toufic Kachaamy, MD

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1 Accepted Manuscript Classical features of Zollinger-Ellison syndrome, in images Ali Alshati, MD, Toufic Kachaamy, MD PII: S (19) DOI: Reference: YMGE To appear in: Gastrointestinal Endoscopy Received Date: 10 December 2018 Accepted Date: 30 January 2019 Please cite this article as: Alshati A, Kachaamy T, Classical features of Zollinger-Ellison syndrome, in images, Gastrointestinal Endoscopy (2019), doi: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

2 Classical features of Zollinger-Ellison syndrome, in images Ali Alshati, MD 1, Toufic Kachaamy, MD 2 1 Department of Internal Medicine, Maricopa Integrated Health System, Creighton University, Phoenix, AZ, USA 2 Department of Gastroenterology and Advanced Endoscopy, Western Regional Medical Center, Cancer Treatment Centers of America, Goodyear, AZ, USA Corresponding author: Ali Alshati, MD Maricopa Integrated Health System, Creighton University 2601 E Roosevelt St, Phoenix, AZ cell: (832) ali.alshati88@gmail.com

3 Classical features of Zollinger-Ellison syndrome, in images A 64-year-old man presented with watery diarrhea and 40-pound weight loss. He has a history of gastroesophageal reflux disease and esophagitis seen in upper endoscopy done previously. His epigastric discomfort and heartburn symptoms were getting worse with eating and were poorly responsive a daily dose of 40 mg oral omeprazole. The diarrhea was not improving in response to fasting. He denied dysphagia or odynophagia. There was no evidence of gastrointestinal infection. He has no history of nonsteroidal anti-inflammatory drug ingestion. Laboratory workup showed fasting gastrin level 669 pg/ml, serum chromogranin A 100 ng/l and gastric PH at 1. A CT scan with contrast of the abdomen and pelvis showed a normal pancreas. However, it showed multiple, solid with cystic component liver lesions that are compatible with metastatic disease. CT-guided core-needle liver biopsy showed metastatic well-differentiated neuroendocrine carcinoma/carcinoid tumor. The primary tumor was undetermined. Upper endoscopy showed diffuse erosive inflammation of the second part of the duodenum (Figure A). Closer endoscopic look under water magnification showed the hypertrophied parietal cells (Figure B). Duodenal biopsy showed findings consistent with chronic peptic duodenitis with no evidence of dysplasia or malignancy. EUS was ordered to look for pancreatic gastrinomas after completing the CT-guided liver biopsy. EUS demonstrated a single, well-defined 2x2, round, hypoechoic, heterogeneous solid mass in the head of the pancreas (Figure C, left, yellow arrow). The outer margin of the mass was slightly irregular. EUS also showed a gastric wall thickening (Figure C, right, yellow arrows). Beside the physiologic uptake in the kidneys and the spleen, the pentetreotide scan demonstrated intense focal uptake in a 2.3 cm soft tissue related to the ventral surface of the pancreatic head, (Figure D, red arrows), consistent with malignancy. It also showed multiple radiotracer avid liver metastases. The patient was treated with long-acting octreotide 30 mg and experienced relief of symptoms.

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