Ménétrier's disease

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Ménétrier's disease
Diagnosis in short

Biopsy compatible with Ménétrier's disease. H&E stain.

Synonyms diffuse foveolar cell hyperplasia, hypertrophic protein-losing gastropathy,

LM marked foveolar cell hyperplasia, decreased parietal cells, +/-inflammation
LM DDx Cronkhite-Canada syndrome, hyperplastic polyp of the stomach
Gross "bag of worms" appearance - very thick gastric folds
Site stomach

Associated Dx gastric adenocarcinoma
Signs emesis (intractible), peripheral edema
Symptoms abdominal pain
Prevalence extremely rare
Endoscopy very thick gastric folds
Clin. DDx gastric cancer

Ménétrier's disease is a very rare pathology of the stomach. It is also known as diffuse foveolar cell hyperplasia,[1] and hypertrophic protein-losing gastropathy.[2][3]

General

Clinical:[4]

  • Classical: nausea, emesis, abdominal pain and peripheral edema.
    • Emesis (intractable) - most important.

Other:

  • Gastric mass (may mimic cancer).
  • Hypochlorhydria.
  • Protein loss (hypoalbuminemia) - leads to peripheral edema.

Epidemiology:

Treatment:

  • EGFR inhibitors.[5]
  • Gastrectomy.

Note:

  • ‡ A similar constellation of symptoms can be seen in children and may be associated with CMV infection.[3][6]

Gross

  • "Bag of worms" appearance - very thick gastric folds.

Images

Microscopic

Features:[1]

  • Foveolar cell hyperplasia - key feature.
  • Decreased parietal cells.
  • +/-Inflammation.

DDx:

Images

www:

See also

References

  1. 1.0 1.1 1.2 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 410. ISBN 978-1416054542.
  2. Hillman, MM.; Meinarde, LL.; Furnes, RA.; Daruich, ML.; Riva, V.; Cuestas, E. (Oct 2013). "[Protein losing gastroenteropathy and possible relationship to cytomegalovirus infection: Ménétrier disease in a child].". Arch Argent Pediatr 111 (5): 446-9. doi:10.1590/S0325-00752013000500016. PMID 24092036.
  3. 3.0 3.1 Meuwissen, SG.; Ridwan, BU.; Hasper, HJ.; Innemee, G. (1992). "Hypertrophic protein-losing gastropathy. A retrospective analysis of 40 cases in The Netherlands. The Dutch Ménétrier Study Group.". Scand J Gastroenterol Suppl 194: 1-7. PMID 1298040.
  4. Rich, A.; Toro, TZ.; Tanksley, J.; Fiske, WH.; Lind, CD.; Ayers, GD.; Piessevaux, H.; Washington, MK. et al. (Dec 2010). "Distinguishing Ménétrier's disease from its mimics.". Gut 59 (12): 1617-24. doi:10.1136/gut.2010.220061. PMID 20926644.
  5. Toubia, N.; Schubert, ML. (Apr 2008). "Menetrier's Disease.". Curr Treat Options Gastroenterol 11 (2): 103-8. PMID 18321437.
  6. Kindermann, A.; Koletzko, S. (Feb 1998). "[Protein-losing giant fold gastritis in childhood--a case report and differentiation from Ménétrier disease of adulthood].". Z Gastroenterol 36 (2): 165-71. PMID 9544500.
  7. Junnarkar SP, Sloan JM, Johnston BT, Laird JD, Irwin ST (May 2001). "Cronkhite-Canada syndrome". The Ulster medical journal 70 (1): 56–8. PMC 2449205. PMID 11428328. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2449205/.
  8. Friedman, J.; Platnick, J.; Farruggia, S.; Khilko, N.; Mody, K.; Tyshkov, M.. "Ménétrier disease.". Radiographics 29 (1): 297-301. doi:10.1148/rg.291075216. PMID 19168850.