Contents
Introduction
Rare acquired disorder characterized by giant gastric rugal folds in the body and fundus, often with antral sparing, decreased acid secretion, increased gastric mucus production, and hypoalbuminemia secondary to protein loss in the gastric mucosa.
- Also known as hyperplastic hypersecretory gastropathy or giant hypertrophic gastritis
- Premalignant condition with an insidious onset with progressive features and is associated with an increased risk of gastric cancer.
History
In 1888 Ménétrier first described the disorder while performing post-mortem studies, noticing hyperplastic changes of the gastric mucosa in cadavers. At the time, he named the disease polyadenomes en nappe. Although Ménétrier understood the debilitative factors of the disease, it wouldn’t be until years later that the associated protein-losing enteropathic aspects of the disorder were realized. Other names for “Ménétrier’s disease” are “hyperplastic hypersecretory gastropathy” and “giant hypertrophic gastritis“. He published his findings in a treatise titled Des polyadenomes gastriques et de leurs rapports avec le cancer de l’estomac.
Pathophysiology
- Gastric mucosal hyperplasia → Hypertrophied rugae → ↑ mucus production & resultant protein loss
- Parietal cell atrophy → ↓ Acid production↑
Clinical presentation
WAVEE:
- Weight loss
- Anorexia
- Vomiting
- Epigastric pain
- Peripheral oedema (due to hypoalbuminemia)
Diagnosis
Endoscopy:
CT-scan:
Differential diagnosis:
| Diagnosis | Distribution | Location in stomach | Hyperplastic mucosal compartment | Pathologic features |
|---|---|---|---|---|
| Ménétrier’s disease | Diffuse | Body and fundus; relative sparing of antrum | Foveolar epithelium | Massive foveolar hyperplasia |
| Hypertrophic lymphocytic gastritis | Diffuse | Body and fundus; relative sparing of antrum | Foveolar epithelium | Prominent intraepithelial lymphocytes |
| Hypertrophic hypersecretory gastropathy | Diffuse | Body and fundus; atrophic antrum | All layers | Hyperplasia of all glandular compartments |
| Zollinger-Ellison syndrome | Diffuse | Body and fundus | Parietal cells | Parietal cell hyperplasia |
| Hyperplastic polyp | Focal | Antrum; body and fundus also possible | Foveolar epithelium | Foveolar hyperplasia with architectural distortion |
| Polyposis syndrome with hamartomatous polyps | Variable | Body, fundus, and antrum | Foveolar epithelium | Features similar to hyperplastic polyp |
| Gastric adenocarcinoma and proximal polyposis of the stomach | Variable | Body and fundus | Oxyntic glands | Fundic gland polyps with low and high-grade dysplasia |
| Diffuse gastric carcinoma | Variable | Body, fundus, and antrum | Not applicable | Infiltrating carcinoma; diffuse type |
| Lymphoma | Variable | Body, fundus, and antrum | Not applicable | Effacement of gastric mucosa by infiltrating lymphoma cells |
| Amyloidosis | Variable | Body, fundus, and antrum | Not applicable | Acellular, amorphous eosinophilic material surrounding glands and vessels |
Management
- Cetuximab