P2543 - A Rare Case of Menetrier's Disease

Michelle Bernshteyn, MD¹, Abdul Bhutta, MBBS², Muhammad O. Arif, MD^3
1Upstate Medical University, Syracuse, NY; ²State University of New York Upstate Medical University, Syracuse, NY; ³SUNY Upstate Medical University, Syracuse, NY

Introduction: Menetrier’s Disease is a rare protein-losing enteropathy that is underrecognized amongst clinicians. Although rare, it is important to recognize and include this diagnosis as a differential when multiple masses are seen.

Case Description/Methods: 41-year-old male with history of recently fatty liver versus cirrhosis with esophageal varices, cerebrovascular accident, mood disorder, hypertension, splenic infarction, and polysubstance use with an incidental gastric mass on imaging and colonic mass seen on colonoscopy.

Endoscopy demonstrated large fungating and ulcerated masses in the stomach and esophagus. Biopsies were benign but determined that deeper biopsies were needed. In the interim, the patient was admitted for hemodynamically insignificant hematemesis. Imaging demonstrated concern for gastric outlet obstruction. Endoscopy showed new mucosal changes in the duodenum. Biopsy of a gastric polyp was suggestive of Menetrier’s Disease. No surgical option due to polyp burden.

Lab studies notable for elevated chromogranin A 2,775 ng/mL (low specificity for carcinoid syndrome), calcitonin 15.5 pg/mL, albumin 2.1 g/dL, and gastrin 318 pg/mL. Cytomegalovirus (CMV), serum H. pylori, alpha-1 anti-trypsin, ceruloplasmin, fibrosure, HIV, and syphilis were negative.

The patient was treated with a proton-pump inhibitor twice daily, Octreotide 100 mcg four times daily, and Cetuximab infusion. Bidirectional scope to correlate masses was delayed to treat acute upper extremity thrombus. Gastric and duodenal polyps on endoscopy with regression of esophageal mass. Sigmoid polyp removed in colonoscopy suspicious for adenocarcinoma.

Discussion: Menetrier’s is a rare disease from increased transdermal growth factor-a expression and heightened epidermal growth factor receptor (EGFR) activity. It can present with hematemesis. It is a protein-losing gastroenteropathy with impressive thickening of gastric mucosa. Histological features include gland dilation and gastric pit expansion. Labs show elevated gastrin and low albumin. CMV oftentimes co-exists in the pediatric population. Octreotide can control EGFR signaling but Cetuximab has been demonstrated to cause remission.

Patients are at risk for protein-loss, gastric outlet, small bowel, and esophageal obstruction. Interestingly, there has been report of acquired thrombophilia as a direct result. Our patient developed an acute upper extremity deep venous thrombus which may be linked to Menetrier’s Disease. Thus, it's important for clinician awareness of this disease.


Figure: A. Gastric body.
B. Middle third of the esophagus, prior to treatment with Cetuximab, demonstrating a mass.
C. Second portion of the duodenum on initial endoscopy.
D. Second portion of the duodenum on follow up endoscopy demonstrating progression of disease.
E. Middle third of the esophagus demonstrating resolution of mass after treatment with Cetuximab.
F. Polyp of the sigmoid colon demonstrated on colonoscopy.


Disclosures:
Michelle Bernshteyn indicated no relevant financial relationships.
Abdul Bhutta indicated no relevant financial relationships.
Muhammad Arif indicated no relevant financial relationships.


Michelle Bernshteyn, MD¹, Abdul Bhutta, MBBS², Muhammad O. Arif, MD³. P2543 - A Rare Case of Menetrier's Disease, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.