What is the recommended surveillance and management for patients with Menetrier disease to reduce the risk of gastric cancer?

Surveillance and Management of Ménétrier's Disease to Reduce Gastric Cancer Risk

Patients with Ménétrier's disease should undergo regular endoscopic surveillance every 3 years due to the significant risk of gastric cancer development, reported at approximately 8.9% at 10 years after diagnosis. 1

Understanding Ménétrier's Disease and Cancer Risk

Ménétrier's disease is a rare acquired disorder characterized by:

• Giant gastric folds
• Protein-losing enteropathy
• Low stomach acid or achlorhydria
• Massive foveolar hyperplasia on histology

The disease carries a substantial risk of malignant transformation:

• 8.9% of patients develop gastric cancer within 10 years of diagnosis 1
• Historical literature suggests a 10-15% risk of gastric cancer development 2
• Multiple case reports document the association between Ménétrier's disease and gastric adenocarcinoma 3, 4, 5
• Patients with Ménétrier's disease have reduced survival rates (72.7% at 5 years and 65.0% at 10 years) compared to controls 1

Recommended Surveillance Protocol

Based on the British Society of Gastroenterology guidelines for patients at risk of gastric adenocarcinoma 6:

1. Initial Assessment:

   • High-quality endoscopy with image-enhanced endoscopy (IEE) where available
   • Systematic biopsies following the Sydney protocol
   • Testing for H. pylori with eradication if positive

2. Surveillance Interval:

   • Endoscopic surveillance every 3 years
   • This recommendation aligns with the BSG guidelines for extensive gastric atrophy or intestinal metaplasia 6

3. Endoscopic Technique:

   • Use high-resolution image-enhanced endoscopy (IEE) when available
   • Document the location and extent of abnormalities with photographic evidence
   • Obtain directed biopsies from concerning areas and random biopsies following the Sydney protocol
   • Samples should be collected in separate containers and labeled appropriately 6

4. Risk Stratification:

   • Document the distribution of giant folds (corpus-predominant disease may carry higher risk)
   • Assess for presence of dysplasia, which requires more intensive surveillance
   • If low-grade dysplasia is found, repeat endoscopy within 1 year 6
   • If high-grade dysplasia is found, repeat endoscopy at 6-month intervals 6

Management of Detected Abnormalities

1. Dysplasia Management:

   • All gastric dysplasia should be resected en bloc when possible 6
   • Endoscopic mucosal resection (EMR) for lesions ≤10 mm
   • Endoscopic submucosal dissection (ESD) for lesions >10 mm

2. Early Gastric Cancer:

   • Complete (R0) endoscopic resection may be considered curative for early gastric adenocarcinoma meeting specific criteria 6
   • Referral to a multidisciplinary team with expertise in gastric cancer is essential

Additional Considerations

1. H. pylori Testing and Eradication:

   • All patients with Ménétrier's disease should be tested for H. pylori and treated if positive 6
   • Note that H. pylori infection is not typically associated with Ménétrier's disease (only 2.6% of patients vs 4.0% of controls) 1

2. Lifestyle Modifications:

   • Smoking cessation should be strongly encouraged as smoking is associated with Ménétrier's disease 1
   • Alcohol use has not been associated with Ménétrier's disease 1

3. Monitoring for Complications:

   • Regular monitoring of protein levels due to protein-losing enteropathy
   • Assessment of nutritional status and weight

Pitfalls and Caveats

1. Diagnostic Challenges:

   • Ménétrier's disease can be difficult to diagnose and may require multiple biopsies
   • Ensure adequate sampling of the gastric mucosa to avoid missing focal areas of dysplasia or early cancer

2. Surveillance Limitations:

   • Standard white light endoscopy has poor accuracy for detecting early neoplastic changes
   • Image-enhanced endoscopy should be used when available to improve detection

3. Risk Assessment:

   • The risk of gastric cancer in Ménétrier's disease may be underestimated due to its rarity
   • The significant mortality difference between patients with Ménétrier's disease and controls (65% vs 100% 10-year survival) underscores the importance of surveillance 1

The evidence supports that Ménétrier's disease should be considered a precancerous condition requiring regular endoscopic surveillance to reduce morbidity and mortality from gastric cancer.