S1877 Treatment Refractory Esophageal Ulceration and Stricture: An Interesting Presentation of Zollinger-Ellison Syndrome

Abstract

INTRODUCTION: Esophageal symptoms are common in patients with Zollinger Ellison syndrome, however, esophageal stricture is found in only a small percentage of patients. We present a case of severe erosive esophagitis with recurrent esophageal stricture refractory to standard medical therapy due to Zollinger Ellison syndrome. CASE DESCRIPTION/METHODS: A 72-year-old male with a medical history significant for HIV on HAART, GERD, hypertension, H. pylori treated with quadruple therapy in 2017, and chronic pancreatitis secondary to remote alcohol abuse presented to the ED with worsening epigastric pain associated with nausea, vomiting, and diarrhea. Stool testing was positive for campylobacter and enteropathogenic E. coli, and H. pylori stool antigen was positive. EGD showed severe erosive esophagitis and multiple duodenal ulcers. Gastrin level was 437 pg/mL, however, the patient was not fasting and was on proton pump inhibitor therapy. The patient completed quadruple therapy for H. pylori and supportive care for presumed infectious diarrhea. He was continued on twice daily PPI therapy after completing treatment for H. pylori. The patient presented back to the ED approximately one month later with dysphagia. EGD showed severe erosive esophagitis and luminal narrowing in the mid-esophagus (approximately 2–3 mm). Repeat gastrin level was 927 pg/mL. PET scan demonstrated a DOTATE avid mass in the first portion of the duodenum consistent with a gastrinoma. The patient was placed on maximal medical therapy (pantoprazole 240 mg/day, famotidine 80 mg/day); however, required serial dilation every 1–2 weeks for recurrent esophageal stricture. He eventually underwent distal gastrectomy and duodenal resection for removal of the gastrin secreting tumor. The final pathology report noted a grade 1 well-differentiated neuroendocrine tumor in the duodenum. DISCUSSION: Zollinger Ellison syndrome is caused by the secretion of gastrin by neuroendocrine tumors (i.e., gastrinomas) most commonly found in the duodenum and pancreas. It most often presents with peptic ulcer disease, heartburn, and diarrhea. Although 31–56% of patients present with esophageal symptoms, esophageal stricture is found in only 4–10% of patients. Our patient was found to have Zollinger Ellison syndrome after presenting with treatment refractory esophageal ulceration and stricture. Medical therapy is the current standard of care, however, surgical resection and other systemic therapy is sometimes required to control symptoms and tumor growth.Figure 1.: Endoscopic images showing severe erosive esophagitis in the proximal esophagus.Figure 2.: Endoscopic images showing luminal narrowing in the mid-esophagus.Figure 3.: Barium esophagram demonstrating severe, long segment stricture of the mid to distal esophagus.