George Washington University Washington, DC, United States
Award: Presidential Poster Award
Ali A. Khan, MD, MPH1, Kerian Dodds, MD2, Steven Zeddun, MD1, Sun A A. Kim, MD, PhD3, Marie L. Borum, MD, EdD, MPH4 1George Washington University, Washington, DC; 2George Washington University, Alexandria, VA; 3The George Washington University, Washington, DC; 4George Washington University School of Medicine and Health Sciences, Washington, DC
Introduction: Zollinger-Ellison syndrome (ZES) is a rare and life-threatening disease caused by a gastrinoma. The average time from symptom onset to diagnosis is >5 years.
Case Description/Methods: A 42-year-old man presented with 8 months of burning epigastric and lower abdominal pain and non-bloody diarrhea 5 times a day.
He had right sided abdominal tenderness without peritoneal signs. Labs and stool studies were normal. CT abdomen/pelvis showed a 7.2 cm cystic mass of the pancreatic tail with partially calcified wall and small bowel enteritis.
EGD showed severe esophagitis in the entire esophagus and active bleeding in the distal duodenum and large ulcers. Colonoscopy showed melena and clots.
He was put on high dose PPI, and stat CTA showed no active GI bleeding, ulcerations in the small bowel, enteritis, and again a 7.7 cm pancreatic cystic mass with heterogenously enhancing solid mural components and thickened wall along its medial and superior margin.
Repeat EGD/enteroscopy showed significantly improved esophagitis, multiple small clean based ulcers in proximal and distal duodenum, and two large round clean based ulcers, one with a hematin spot, in the proximal jejunum. EUS showed a single 54mm x 44mm cyst in the pancreas tail; FNA of the cyst fluid was performed revealing a thick brown liquid; FNB on the large enhancing cyst wall confirmed gastrinoma. (Figure 1)
Gastrin level was 649, MEN syndromes were ruled out, and DOTATATE PET/CT scan did not show metastasis of known gastrinoma.
He underwent distal pancreatectomy, splenectomy, and small bowel resections. Pathology revealed well differentiated NET with lymphovascular invasion, pancreatic intraepithelial neoplasm, two segments of small intestine with chronic serositis and fibrosis – one of which had multifocal ulcerations.
Discussion: This case demonstrates a timely diagnosis of gastrinoma, which occurred within one week of presentation. The location of the gastrinoma in our patient was atypical: a pancreatic tail cyst. 90% of gastrinomas occur within the gastrinoma triangle; formed by (1) the junction of common and cystic duct, (2) junction of the body and neck of pancreas, and (3) the junction of the 2nd and 3rd portion of the duodenum. EUS guided FNB of the thickened cyst wall confirmed the diagnosis. It is important to maintain ZES on the differential when evaluating abdominal pain and diarrhea to allow for timely diagnosis and improved outcomes.
Figure: Figure 1. (A) Severe esophagitis seen on initial EGD. (B) Improved esophagitis on repeat EGD, after high dose PPI therapy. (C) Clean based ulcer with hematin spot in proximal jejunum. (D) Pancreas tail cyst on EUS. (E) Enhancing cyst wall on EUS. (F) Pathology from FNB showing mitosis and nuclear features of gastrinoma.
Disclosures:
Ali Khan indicated no relevant financial relationships.
Kerian Dodds indicated no relevant financial relationships.
Steven Zeddun indicated no relevant financial relationships.
Sun A Kim indicated no relevant financial relationships.
Marie Borum indicated no relevant financial relationships.
Ali A. Khan, MD, MPH1, Kerian Dodds, MD2, Steven Zeddun, MD1, Sun A A. Kim, MD, PhD3, Marie L. Borum, MD, EdD, MPH4. P1730 - Think Outside the Triangle: Timely Diagnosis of a Gastrinoma in a Pancreatic Cyst, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.
