University of Alabama at Birmingham Birmingham, AL
Award: Presidential Poster Award
Yassmin Hegazy, MD, Dane Johnson, MD, Jim McPhail, MD, Douglas Morgan, MD, MPH University of Alabama at Birmingham, Birmingham, AL
Introduction: A 64-year-old female with a history of diabetes mellitus presents with acute on chronic diarrhea and refractory acid reflux is found to have Zollinger-Ellison syndrome.
Case Description/Methods: The patient had a two-year history of non-bloody diarrhea which worsened (7 bowel movements/day) in the two months prior to presentation. She described associated nausea, reflux, and abdominal pain. Notably, her mother had similar symptoms, requiring small bowel resection (records not available). Previous workup included a colonoscopy and an esophagoduodenoscopy (EGD) showing duodenal ulcerations with negative biopsies for celiac disease. She was started on a proton-pump inhibitor (PPI), colestipol, and loperamide without improvement. Medication review, stool infectious studies, and cross-sectional imaging had been unrevealing.
On presentation, she had stable vitals and physical exam significant for diffuse abdominal tenderness. Labs were notable for a hemoglobin of 10.2 gm/dL (grams per deciliter) and an acute kidney injury (AKI). Abdominal Computed Tomography (CT) suggested multiple, small duodenal ulcers with a polypoid lesion in the gastric antrum (Figure 1). EGD revealed esophagitis, multiple small duodenal ulcerations (D1, D2), and gastric mucosal atrophy. Repeat colonoscopy was unremarkable. The fasting gastrin level was 211 pg/mL (picogram per milliliter). The secretin stimulation test showed serum gastrin blood levels of 121, 258, and 483 pg/mL at 2, 5, and 10 minutes respectively consistent with Zollinger-Ellison Syndrome. The Dotatate PET/CT scan showed metastatic somatostatin receptor expressing portocaval adenopathy without a tracer-avid primary neuroendocrine tumor. She was started on lanreotide and is now referred to oncology.
Discussion: This case represents an instructive example of Zollinger Ellison Syndrome (ZES), often discussed, but rarely diagnosed. In this case, the patient presented with progressive diarrhea, a subtle family history, and with several prior evaluations. ZES is a rare gastrin-secreting neuroendocrine tumor (NET) associated with multiple endocrine neoplasia (MEN-1), which leads to refractory acid reflux and chronic diarrhea, as seen in our patient. Non-specific gastrointestinal symptoms and empiric PPI use can delay the diagnosis. The gastric pH, gastric level, secretin stimulation, and Dotatate PET/CT are used to evaluate and localize the tumor.
Figure: Figure 1A, 1B: Computed Tomography (CT) abdomen showed multiple, small duodenal ulcers and 4 millimeter (mm) enhancing polyp at the gastric antrum (red arrows)
Disclosures:
Yassmin Hegazy indicated no relevant financial relationships.
Dane Johnson indicated no relevant financial relationships.
Jim McPhail indicated no relevant financial relationships.
Douglas Morgan: Cancer Prevention Pharmaceuticals – NCI funded study, company donated study medications.. CDx Diagnostics – Investigator initiated study, company donated disposables.. Freenome – Grant/Research Support. Thorne Research – NCI funded study, company donated study medications..
Yassmin Hegazy, MD, Dane Johnson, MD, Jim McPhail, MD, Douglas Morgan, MD, MPH. B0666 - A Case of Progressive Refractory Diarrhea due to Zollinger-Ellison Syndrome, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.
