Difference between revisions of "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'''.<ref name=Ref_PCPBoD8_410>{{Ref PCPBoD8|410}}</ref>
'''Ménétrier's disease''' is a very rare pathology of the [[stomach]]. It is also known as '''diffuse foveolar cell hyperplasia''',<ref name=Ref_PCPBoD8_410>{{Ref PCPBoD8|410}}</ref> and '''hypertrophic protein-losing gastropathy'''.<ref name=pmid24092036>{{Cite journal  | last1 = Hillman | first1 = MM. | last2 = Meinarde | first2 = LL. | last3 = Furnes | first3 = RA. | last4 = Daruich | first4 = ML. | last5 = Riva | first5 = V. | last6 = Cuestas | first6 = E. | title = [Protein losing gastroenteropathy and possible relationship to cytomegalovirus infection: Ménétrier disease in a child]. | journal = Arch Argent Pediatr | volume = 111 | issue = 5 | pages = 446-9 | month = Oct | year = 2013 | doi = 10.1590/S0325-00752013000500016 | PMID = 24092036 }}</ref><ref name=pmid1298040>{{Cite journal  | last1 = Meuwissen | first1 = SG. | last2 = Ridwan | first2 = BU. | last3 = Hasper | first3 = HJ. | last4 = Innemee | first4 = G. | title = Hypertrophic protein-losing gastropathy. A retrospective analysis of 40 cases in The Netherlands. The Dutch Ménétrier Study Group. | journal = Scand J Gastroenterol Suppl | volume = 194 | issue =  | pages = 1-7 | month =  | year = 1992 | doi =  | PMID = 1298040 }}</ref>


==General==
==General==
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Epidemiology:
Epidemiology:
*Men > women.
*Men > women.
*Adults usually 50s.
*Adults usually 50s.
*Associated with [[ulcerative colitis]].
*Associated with [[ulcerative colitis]].


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*EGFR inhibitors.<ref name=pmid18321437>{{Cite journal  | last1 = Toubia | first1 = N. | last2 = Schubert | first2 = ML. | title = Menetrier's Disease. | journal = Curr Treat Options Gastroenterol | volume = 11 | issue = 2 | pages = 103-8 | month = Apr | year = 2008 | doi =  | PMID = 18321437 }}</ref>
*EGFR inhibitors.<ref name=pmid18321437>{{Cite journal  | last1 = Toubia | first1 = N. | last2 = Schubert | first2 = ML. | title = Menetrier's Disease. | journal = Curr Treat Options Gastroenterol | volume = 11 | issue = 2 | pages = 103-8 | month = Apr | year = 2008 | doi =  | PMID = 18321437 }}</ref>
*Gastrectomy.
*Gastrectomy.
Note:
*‡ A similar constellation of symptoms can be seen in children and may be associated with [[CMV]] infection.<ref name=pmid1298040/><ref>{{Cite journal  | last1 = Kindermann | first1 = A. | last2 = Koletzko | first2 = S. | title = [Protein-losing giant fold gastritis in childhood--a case report and differentiation from Ménétrier disease of adulthood]. | journal = Z Gastroenterol | volume = 36 | issue = 2 | pages = 165-71 | month = Feb | year = 1998 | doi =  | PMID = 9544500 }}</ref>
   
   
==Gross==
==Gross==

Revision as of 02:10, 10 October 2014

Ménétrier's disease
Diagnosis in short

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

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. 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.