Difference between revisions of "Ménétrier's disease"

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#redirect [[Stomach#M.C3.A9n.C3.A9trier.27s_disease]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Ménétrier's disease -- very low mag.jpg
| Width      =
| Caption    = Biopsy compatible with Ménétrier's disease. [[H&E stain]].  
| Synonyms  = diffuse foveolar cell hyperplasia, hypertrophic protein-losing gastropathy,
| Micro      = marked foveolar cell hyperplasia, decreased parietal cells, +/-inflammation
| Subtypes  =
| LMDDx      = [[Cronkhite-Canada syndrome]], [[hyperplastic polyp of the stomach]]
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      = "bag of worms" appearance - very thick gastric folds
| Grossing  =
| Site      = [[stomach]]
| Assdx      = [[gastric adenocarcinoma]]
| Syndromes  =
| Clinicalhx =
| Signs      = emesis (intractible), peripheral edema
| Symptoms  = abdominal pain
| Prevalence = extremely rare
| Bloodwork  =
| Rads      =
| Endoscopy  = very thick gastric folds
| Prognosis  =
| Other      =
| ClinDDx    = [[gastric cancer]]
}}
'''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==
*Super rare.
*Increased risk of [[gastric adenocarcinoma]].<ref name=Ref_PCPBoD8_410>{{Ref PCPBoD8|410}}</ref>
Clinical:<ref name=pmid20926644>{{Cite journal  | last1 = Rich | first1 = A. | last2 = Toro | first2 = TZ. | last3 = Tanksley | first3 = J. | last4 = Fiske | first4 = WH. | last5 = Lind | first5 = CD. | last6 = Ayers | first6 = GD. | last7 = Piessevaux | first7 = H. | last8 = Washington | first8 = MK. | last9 = Coffey | first9 = RJ. | title = Distinguishing Ménétrier's disease from its mimics. | journal = Gut | volume = 59 | issue = 12 | pages = 1617-24 | month = Dec | year = 2010 | doi = 10.1136/gut.2010.220061 | PMID = 20926644 }}</ref>
*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:
*Men > women.
*Adults usually 50s.‡
*Associated with [[ulcerative colitis]].
Treatment:
*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.
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==
*"Bag of worms" appearance - very thick gastric folds.
===Images===
<gallery>
Image:Menetrier.jpg | Ménétrier's disease. (WC/AFIP)
</gallery>
==Microscopic==
Features:<ref name=Ref_PCPBoD8_410>{{Ref PCPBoD8|410}}</ref>
*Foveolar cell hyperplasia - '''key feature'''.
*Decreased parietal cells.
*+/-Inflammation.
DDx:
*[[Cronkhite-Canada syndrome]].<ref name="pmid11428328">{{cite journal |author=Junnarkar SP, Sloan JM, Johnston BT, Laird JD, Irwin ST |title=Cronkhite-Canada syndrome |journal=The Ulster medical journal |volume=70 |issue=1 |pages=56–8 |year=2001 |month=May |pmid=11428328 |pmc=2449205 |doi= |url=}}</ref>
*[[Hyperplastic polyp of the stomach]].
===Images===
<gallery>
Image: Ménétrier's disease -- very low mag.jpg | MD - very low mag. (WC)
Image: Ménétrier's disease -- low mag.jpg | MD - low mag. (WC)
Image: Ménétrier's disease - alt -- low mag.jpg | MD - low mag. (WC)
Image: Ménétrier's disease -- intermed mag.jpg | MD - intermed. mag. (WC)
Image: Ménétrier's disease -- high mag.jpg | MD - high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case36.html Ménétrier's disease - low quality images (upmc.edu)].
*[http://radiographics.rsna.org/content/29/1/297.full.pdf Ménétrier's disease - several images from the AFIP (rsna.org)].<ref name=pmid19168850>{{Cite journal  | last1 = Friedman | first1 = J. | last2 = Platnick | first2 = J. | last3 = Farruggia | first3 = S. | last4 = Khilko | first4 = N. | last5 = Mody | first5 = K. | last6 = Tyshkov | first6 = M. | title = Ménétrier disease. | journal = Radiographics | volume = 29 | issue = 1 | pages = 297-301 | month =  | year =  | doi = 10.1148/rg.291075216 | PMID = 19168850 | URL = http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=19168850 }}</ref>
==See also==
*[[Stomach]].
==References==
{{Reflist|2}}
[[Category:Stomach]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Latest revision as of 22:31, 25 January 2015

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

  • 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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