S3023 Menetrier's Disease Presenting as a Mass in the Cardia of the Stomach

INTRODUCTION: Menetrier’s disease (MD) is a rare acquired protein-losing gastropathy characterized by enlarged gastric folds and hypoalbuminemia. These mucosal folds are generally confined to the parietal cells or oxyntic mucosa in the gastric body and fundus. Herein, we report the first documented case of Menetrier’s disease in the cardia of the stomach. CASE DESCRIPTION/METHODS: A 54 year old man with a history of long standing gastroesophageal reflux disease presented with recalcitrant symptoms despite maximal therapy with proton pump inhibitor (PPI). Initial upper endoscopy showed gastric erythema and heaped up mucosa in the gastric cardia concerning for a mass. He was referred to our institution for endoscopic ultrasonography (EUS) and fine needle aspiration of the gastric cardia mass. Esophagogastroduodenoscopy (EGD) revealed thickened gastric folds throughout, most prominent in the cardia. EUS showed enlarged cardia folds with submucosal thickening (43.6 × 17.5 mm). Biopsy demonstrated predominantly oxyntic mucosa with extensive cystic dilatation of the oxyntic and foveolar lined glands, consistent with Menetrier’s disease. No cytomegalovirus or H. pylori organisms were identified. DISCUSSION: The pathophysiology of Menetrier’s disease is poorly understood but is thought to be due to upregulation of transforming growth factor-alpha (TGF-a) that binds to and enhances the singling of epidermal growth factor receptor (EGFR). TGF-a increases cellular proliferation. EGFR is expressed mainly on the parietal cells of the foveolar or surface mucosal cells. This leads to the proliferation of epithelial cells and hyperplasia of foveolar cells, increasing thick mucous production. The massive foveolar hypertrophy replaces the glandular structures beneath, resulting in atrophic oxyntic glands and a reduction in parietal and chief cells. The loss of acid producing parietal cells increases the gastric pH. These changes ultimately alter the mucosal integrity of the stomach and increases mucosal permeability resulting in a protein-losing enteropathy. Clinical manifestations are nonspecific including dyspepsia, nausea, GI bleeding, and edema. The diagnosis can be challenging as a variety of inflammatory and infiltrative conditions can mimic the large gastric folds of MD, including malignancy. Therefore, large snare or suction biopsies are necessary for diagnosis to capture the full mucosal thickness and document the loss of deep glandular components.Figure 1.: Large hypertrophied gastric folds in the cardia on EGD.Figure 2.: EUS of enlarged gastric folds with submucosal thickness ∼43.6 × 17.5 mm.Figure 3.: Hematoxylin and eosin stain 50x of gastric lesion with prominent foveolar hyperplasia and dilated glands compatible with Menetrier’s disease.