Ménétrier Disease: A Diagnostic Challenge of Enlarged Gastric Folds

Case Report

Figure 1 · Endoscopic findings

Panel A: Retroflexed View Of Enlarged Fundal Gastric Folds
(A) Markedly enlarged and edematous gastric folds in the fundus, with the endoscope visible in retroflexion.
Panel B: Close-Up Of Giant Cerebriform Gastric Folds
(B) Close-up of giant rugal folds with a cerebriform appearance, dark surface staining, and tenacious adherent mucus.
Panel C: Hypertrophic Gastropathy Around The Cardia
(C) Hypertrophic gastropathy around the cardia with foamy mucus pooled between massive folds.

Experienced teaching points

Clinical Pearls

01Keep Ménétrier disease in the differential for giant gastric folds that do not flatten with air insufflation.

02EUS helps confirm intact muscularis propria and lowers the likelihood of infiltrative malignancy before biopsy.

03Deep or full-thickness mucosal sampling is required because the diagnostic histology sits deeper than a superficial pinch biopsy.

04Correct recognition prevents misclassification as gastric cancer or lymphoma and avoids unnecessary oncologic pathways.

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Final Diagnosis

Ménétrier disease diagnosed via endoscopic ultrasound and targeted deep biopsies.

Clinical History

A 76-year-old man with anemia presented for repeat esophagogastroduodenoscopy (EGD) with endoscopic ultrasound (EUS) after a prior EGD showed a large circumferential ulcerated mass at the gastric cardia.

Endoscopic Findings

  1. EGD demonstrated a fundal lesion around the cardia with enlarged and edematous rugal folds.
  2. The folds failed to flatten with air insufflation and were covered by tenacious adherent mucus.
  3. EUS showed intact muscularis propria without invasion into the muscular layer around the fundus.

Endoscopic Technique

Repeat EGD was followed by radial EUS with balloon inflation at the echoendoscope tip to optimize fundal visualization. Multiple deep biopsies of the lesion were obtained.

Pathology. Gastric mucosa with marked foveolar hyperplasia and tortuous gastric pits, focal surface erosion with cystic glandular dilatation toward the base of crypts, variable inflammation, marked mucosal edema, and reduced parietal and chief cells, diagnostic of Ménétrier disease.

Discussion

Mechanism. Ménétrier disease is a rare acquired hypertrophic gastropathy that typically affects men at a mean age of about 55 years. It features giant rugal folds of the body and fundus, hypochlorhydria or achlorhydria, excess mucus production, and protein-losing enteropathy. Clinical manifestations include progressive abdominal pain, nausea, vomiting, and peripheral edema.

What the scope shows. Markedly enlarged, edematous folds that refuse to flatten with air and are coated by tenacious mucus are the endoscopic hallmark. Histology shows massive foveolar hyperplasia, glandular tortuosity and cystic dilation, smooth muscle hyperplasia, and oxyntic atrophy. Because these changes sit deeper in the mucosa, superficial biopsies are often nondiagnostic.

Why it matters. Giant folds also raise concern for infiltrative adenocarcinoma, signet-ring carcinoma, and lymphoma. The differential includes Helicobacter pylori gastritis, Zollinger-Ellison syndrome, portal hypertensive gastropathy, and gastric polyposis syndromes. Intact muscularis propria on EUS plus adequate deep sampling established Ménétrier disease here and helped avoid an unnecessary oncologic pathway.

References

  1. Richer R, Barbu ST. Ménétrier disease: A rare cause of protein-losing gastropathy. Gastrointest Endosc. 2018;88(4):629.
  2. Meuwissen SG, Hasler WL, van der Waaij LA. Ménétrier disease: Clinical, endoscopic, and histopathological features. World J Gastroenterol. 2011;17(3):329-335.
  3. Komorowski RA, Caya JG. Ménétrier disease: A clinicopathologic study of 16 patients. Hum Pathol. 1984;15(3):264-272.

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