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''' | {{ 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== | ==General== | ||
*Super rare. | *Super rare. | ||
*Increased risk of gastric adenocarcinoma.<ref name=Ref_PCPBoD8_410>{{Ref PCPBoD8|410}}</ref> | *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> | 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> | ||
| Line 16: | Line 46: | ||
Epidemiology: | Epidemiology: | ||
*Men > women. | *Men > women. | ||
*Adults usually 50s. | *Adults usually 50s.‡ | ||
*Associated with [[ulcerative colitis]]. | *Associated with [[ulcerative colitis]]. | ||
| Line 22: | Line 52: | ||
*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== | ||
*"Bag of worms" appearance - very thick gastric folds. | *"Bag of worms" appearance - very thick gastric folds. | ||
===Images=== | |||
<gallery> | |||
Image:Menetrier.jpg | Ménétrier's disease. (WC/AFIP) | |||
</gallery> | |||
==Microscopic== | ==Microscopic== | ||
| Line 36: | Line 74: | ||
*[[Hyperplastic polyp of the stomach]]. | *[[Hyperplastic polyp of the stomach]]. | ||
Images: | ===Images=== | ||
*[http://path.upmc.edu/cases/case36.html Ménétrier's disease - | <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== | ==See also== | ||
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
- Super rare.
- Increased risk of gastric adenocarcinoma.[1]
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:
- Men > women.
- Adults usually 50s.‡
- Associated with ulcerative colitis.
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:
- Ménétrier's disease - low quality images (upmc.edu).
- Ménétrier's disease - several images from the AFIP (rsna.org).[8]
See also
References
- ↑ 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.
- ↑ 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.
- ↑ 3.0 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.
- ↑ 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.
- ↑ Toubia, N.; Schubert, ML. (Apr 2008). "Menetrier's Disease.". Curr Treat Options Gastroenterol 11 (2): 103-8. PMID 18321437.
- ↑ 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.
- ↑ 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/.
- ↑ 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.