7/9/15, 23:16Ménétrier's disease - Wikipedia, the free encyclopedia

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Ménétrier disease

Gross specimen of biopsy of stomach in Ménétrier disease. In this case, thesubstantial pit hyperplasia makes the large rugal folds appear to be covered bymyriad polyps resembling hyperplastic polyps. The muscularis propria is the

folded structure at the bottom center.

Classification and external resources

ICD-10 K29.6(http://apps.who.int/classifications/icd10/browse/2015/en#/K29.6)

ICD-9-CM 535.2 (http://www.icd9data.com/getICD9Code.ashx?icd9=535.2)

OMIM 137280 (http://omim.org/entry/137280)

DiseasesDB 8001 (http://www.diseasesdatabase.com/ddb8001.htm)

MeSH D005758 (https://www.nlm.nih.gov/cgi/mesh/2015/MB_cgi?field=uid&term=D005758)

Ménétrier's diseaseFrom Wikipedia, the free encyclopedia

Ménétrier disease (also known ashypoproteinemic hypertrophicgastropathy; named after a Frenchphysician Pierre Eugène Ménétrier,1859–1935), is a rare, acquired,premalignant disease of the stomachcharacterized by massive gastricfolds, excessive mucous productionwith resultant protein loss, and littleor no acid production. The disorder isassociated with excessive secretion oftransforming growth factor alpha(TGF-α).[1]

Contents1 Signs and symptoms2 Cause3 Pathology4 Diagnosis5 Treatment6 References

Signs and symptomsThe mean age when signs and symptoms first appear is 30 to 60 years. Individuals with the disease present withupper abdominal pain, at times accompanied by nausea, vomiting, loss of appetite, and weight loss. Occultgastrointestinal bleed may occur, but overt bleeding is unusual and, when present, is due to superficial mucosalerosion. Twenty to 100% of patients, depending on time of presentation, develop a protein-losing gastropathyaccompanied by low blood albumin and edema.[2]

Symptoms and pathological features of Ménétrier disease in children are similar to those in adults, but diseasein children is usually self-limited and often follows respiratory infection.[3]

The risk of gastric adenocarcinoma is increased in adults with Ménétrier disease.[3][4]

Cause

7/9/15, 23:16Ménétrier's disease - Wikipedia, the free encyclopedia

Page 2 of 3https://en.wikipedia.org/wiki/Ménétrier%27s_disease

The cause of Ménétrier disease is unknown, but it has been associated with CMV infection in children and H.pylori infections in adults.[4] Additionally, increased TGF-α has been noted in the gastric mucosa of patientswith the disease.[1]

PathologyThe stomach is characterized by large, tortuous gastric folds in the fundus and body of the stomach, with antrumgenerally spared, giving the mucosa a cobblestone or cerebriform (brain-like) appearance.[4] Histologically, themost characteristic feature is massive foveolar hyperplasia (hyperplasia of surface and glandular mucouscells).[2] The glands are elongated with a corkscrew-like appearance and cystic dilation is common.Inflammation is usually only modest, although some cases show marked intraepithelial lymphocytosis. Diffuseor patchy glandular atrophy, evident as hypoplasia of parietal and chief cells, is typical.[3]

Although ICD-10 classifies it under "Other gastritis" (K29.6(http://apps.who.int/classifications/icd10/browse/2010/en#/K29.6)), and the lamina propria may contain mildchronic inflammatory infiltrate, Ménétrier disease is not considered a form of gastritis.[2] It is rather consideredas one of the two most well understood hypertrophic gastropathies; the other being Zollinger-Ellisonsyndrome.[3]

DiagnosisThe differential diagnosis of large gastric folds includes Zollinger-Ellison syndrome, cancer, infectiousetiologies (CMV, histoplasmosis, syphilis), and infiltrative disorders such as sarcoidosis.[2]

Large gastric folds are readily detectable by either radiographic (barium meal) or endoscopic methods.Endoscopy with deep mucosal biopsy (and cytology) is required to establish the diagnosis and exclude otherentities that may present similarly. A nondiagnostic biopsy may lead to a surgically obtained full-thicknessbiopsy to exclude malignancy.[2]

Twenty-four-hour pH monitoring reveals hypochlorhydria or achlorhydria, and a chromium-labelled albumintest reveals increased GI protein loss.[4] Serum gastrin levels will be within normal limits.

TreatmentMedical therapy with anticholinergic agents, prostaglandins, proton pump inhibitors, prednisone, and H2receptor antagonists yields varying results. Anticholinergics decrease protein loss. A high-protein diet should berecommended to replace protein loss in patients with hypoalbuminemia. Ulcers should be treated with thestandard approach. Severe disease with persistent and substantial protein loss may require total removal of thestomach. Subtotal gastrectomy is performed by some; it may be associated with higher morbidity and mortalitysecondary to the difficulty in obtaining a patent and long-lasting anastomosis between normal and hyperplastictissue. In adults, there is no FDA approved treatment other than gastrectomy and a high-protein diet.

7/9/15, 23:16Ménétrier's disease - Wikipedia, the free encyclopedia

Page 3 of 3https://en.wikipedia.org/wiki/Ménétrier%27s_disease

CT abdomen, coronal section, showingcharacteristic large rugal folds in the stomach. Acyst is also seen in the liver.

More recently, agents that block TGF-α-mediated activationof the epidermal growth factor receptor (EGFR) have shownpromise. Cetuximab, a monoclonal antibody against EGFR,has been used in compassionate use for treatment ofMénétrier's disease.[5]

Pediatric cases are normally treated for symptoms with thedisease clearing up in weeks to months.

References1. Coffey RJ et al.: Menetrier disease and gastrointestinal stromal

tumors: hyperproliferative disorders of the stomach(http://www.jci.org/articles/view/30491). J Clin Invest.2007;117(1):70–80. doi:10.1172/JCI30491

2. Harrison's Principle of Internal Medicine, 18e, pg 24593. Robbins and Cotran, Pathological Basis of Disease, 8e, pg 7824. Townsend et al., Sabiston Textbook of Surgery, 18e, pg 12725. Burdick JS, Chung E, Tanner G et al. (December 2000). "Treatment of Ménétrier's disease with a monoclonal antibody

against the epidermal growth factor receptor" (http://www.nejm.org/doi/full/10.1056/NEJM200012073432305). N. Engl.J. Med. 343 (23): 1697–701. doi:10.1056/NEJM200012073432305(https://dx.doi.org/10.1056%2FNEJM200012073432305). PMID 11106719(https://www.ncbi.nlm.nih.gov/pubmed/11106719).

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Categories: Stomach disorders Hypertrophic gastropathies

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