University of Maryland Medical Center Midtown Campus Baltimore, MD
Mohammed Rifat Shaik, MBBS1, Nishat Anjum Shaik, MBBS2, Erika Wheeler, MD3, Yuting Huang, MD, PhD1, Robert T. Chow, MD, MBA1 1University of Maryland Medical Center Midtown Campus, Baltimore, MD; 2Guntur Medical College, Guntur, Andhra Pradesh, India; 3University of Maryland Medical Center, Baltimore, MD
Introduction: Zollinger Ellison syndrome (ZES) is characterized by gastric hyperacidity, resulting from increased gastrin production. This leads to symptoms such as abdominal pain, heartburn, and diarrhea. Given the overlap in the symptoms with idiopathic Peptic Ulcer Disease (PUD) and Gastro-Esophageal Reflux Disease (GERD), the diagnosis can be delayed and complications such as gastric obstruction, hemorrhage, or even perforation can be the initial presentation. Here we present an unusual case of esophageal perforation that was ultimately diagnosed with ZES.
Case Description/Methods: A 65-year-old male with a history of perforated gastric ulcer s/p Graham patch repair two months prior presented with worsening abdominal pain, vomiting, and inability to swallow secretions. He had a positive H. pylori stool antigen test during the prior hospitalization s/p course of eradication therapy. CT chest showed pneumomediastinum and an esophageal perforation. An upper gastrointestinal endoscopy, done to place an esophageal stent, revealed multiple ulcers in the first portion of the duodenum. Robotic-assisted drainage of the posterior mediastinum was then pursued. Abdominal imaging (CT/MRI) demonstrated enhancing masses in multiple liver segments as well as periportal adenopathy (Fig 1, 2). FDG PET/CT showed increased uptake by the left liver lesion and multiple porta hepatis adenopathy, concerning for metastatic disease. The primary tumor could not be clearly delineated. A percutaneous liver biopsy revealed a metastatic, grade 1 well-differentiated neuroendocrine tumor (Fig 3, 4). Serum gastrin levels were elevated to 433 pg/mL. Serum ionized calcium and parathyroid hormone were within normal limits. Family history was negative for multiple endocrine neoplasia 1 (MEN1). A diagnosis of metastatic, sporadic ZES with an unknown primary site was made. The patient was discharged on high-dose lansoprazole and somatostatin analog with outpatient follow-up for DOTATATE scan and definitive treatment.
Discussion: Peptic ulcer perforation is seen in 4-6 % of the patients with ZES. Most perforations involved the duodenum with one report of jejunal involvement and no reports of gastric perforation to date. There is evidence of an increase in esophageal involvement, with several cases of esophagitis and Barrett’s esophagus; however, very few cases of esophageal perforation were reported. This case is unique in that our patient developed complications rarely described in literature i.e. gastric perforation and esophageal perforation.
Figure: Figure 1 : CT abdomen showing 3.5 cm mass inseparable from segment II of the liver Figure 2 : MRI abdomen showing multiple enhancing masses in segments I, II, and IVA with subtle washout and associated periportal adenopathy most compatible with metastatic disease process Figure 3 : This image of a liver core needle biopsy shows the transition from liver parenchyma (upper right) to the tumor (lower left). The nested pattern of the tumor is suggestive of possible neuroendocrine origin. Figure 4 : Chromogranin (left) and synaptophysin (right) immunohistochemical stains positively highlight the tumor cells (brown) and do not stain the liver parenchyma, confirming neuroendocrine tumor. The lack of pleomorphism, mitotic figures, and low Ki-67 proliferation index (~25, not shown) identify this as a well-differentiated neuroendocrine tumor.
Disclosures:
Mohammed Rifat Shaik indicated no relevant financial relationships.
Nishat Anjum Shaik indicated no relevant financial relationships.
Erika Wheeler indicated no relevant financial relationships.
Yuting Huang indicated no relevant financial relationships.
Robert Chow indicated no relevant financial relationships.
Mohammed Rifat Shaik, MBBS1, Nishat Anjum Shaik, MBBS2, Erika Wheeler, MD3, Yuting Huang, MD, PhD1, Robert T. Chow, MD, MBA1. D0252 - An Unusual Case of Esophageal Perforation, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.
