Ménétrier's disease

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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]

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

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

  • Ménétrier's disease. (WC/AFIP)

Microscopic

Features:[1]

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

DDx:

Images

  • MD - very low mag. (WC)

  • MD - low mag. (WC)

  • MD - low mag. (WC)

  • MD - intermed. mag. (WC)

  • MD - high mag. (WC)

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. https://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.
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