P2079 - A Case of Ménétrier's Disease Treated With Cetuximab

Hannah Ramrakhiani, ¹, George Triadafilopoulos, MD, FACG^2
1Palo Alto Senior High School, Palo Alto, CA; ²Stanford University School of Medicine, Stanford, CA

Introduction: Ménétrier's disease is a rare disorder characterized by giant gastric folds that are the result of hypertrophy of gastric mucosal cells (foveola). We present a case of Ménétrier's disease diagnosed with endoscopic biopsies and treated with cetuximab.

Case Description/Methods: A 65-year old male presented with symptoms of diarrhea, loss of appetite, and significant weight loss. The patient was underweight on initial evaluation (105 lbs). Laboratory studies were significant for hypoalbuminemia (1.1 g/dL). CT scan (fig. 1) and MRI of the abdomen (fig. 2) showed gastric antrum wall thickening. Upper endoscopy and endoscopic ultrasound (fig. 3 and 4) revealed markedly thickened polypoid folds in the stomach with a large amount of overlying mucoid material. Forceps biopsies showed foveolar hyperplasia in the stomach. Because of clinical concern for a hypertrophic gastric polypoid condition, repeat endoscopy was performed with larger snare biopsies and revealed proliferation of foveolar epithelium resulting in elongated, branched pits and dilated glands (fig. 5). In the context of clinical and endoscopic findings, the histologic findings were thought to be compatible with a diagnosis of Ménétrier's disease. Patient was started on sandostatin and total parenteral nutrition (TPN) with initial improvement, however, symptoms worsened a few months later resulting in hospitalizations for dehydration, dyselectrolytemia, and weight loss. He was subsequently referred to medical oncology and started on cetuximab. The patient’s diarrhea and stool frequency decreased by over fifty percent and he was discharged from the skilled nursing facility. During the last visit, the patient had gained a significant amount of weight.

Discussion: Ménétrier's disease is characterized by giant mucosal folds in the stomach causing protein loss. Our case highlights the importance of obtaining larger biopsy specimens with snare resection and clinical pathologic correlation in order to make a diagnosis. Early referral to a nutrition team for consideration of TPN can help prevent major complications like protein calorie malnutrition, dehydration, dyselectrolytemia. Cetuximab, a monoclonal antibody that blocks epidermal growth factor signalling, has shown clinical benefit in some patients. For individuals with severe disease at presentation, oncology consultation should be obtained to assess the need for treatment with cetuximab.


Figure: Fig. 1: CT scan (axial) showing marked thickening of the gastric wall
Fig. 2: MRI (coronal) showing thickening and polypoid appearance of the gastric antrum
Fig. 3: Endoscopic image with thickened gastric folds and overlying mucus
Fig. 4: Endoscopic ultrasound demonstrating marked polypoid thickening of the gastric wall
Fig. 5: Photomicrograph showing proliferation of foveolar epithelium resulting in elongated, branched pits and dilated glands


Disclosures:
Hannah Ramrakhiani indicated no relevant financial relationships.
George Triadafilopoulos indicated no relevant financial relationships.


Hannah Ramrakhiani, ¹, George Triadafilopoulos, MD, FACG². P2079 - A Case of Ménétrier's Disease Treated With Cetuximab, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.