Ménétrier's disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Ménétrier's disease (also known as hyperplastic hypersecretory gastropathy and giant hypertrophic gastropathy and named after a French physician Pierre Eugène Ménétrier, 1859-1935), is a disorder in which the gastric mucosal folds (rugae) are enlarged[1] (and the total weight of the stomach is increased), making the surface of the stomach look a bit like the brain gyri. The altered gastric mucosa secretes massive amounts of mucus, resulting in low plasma protein levels. The tissue may be inflamed and may contain ulcers. The disease also causes glands in the stomach to waste away and causes the body to lose fluid containing a protein called albumin.

There are two forms of the disease: a childhood form and an adult form. The childhood form has a better prognosis. It affects boys and girls equally, most often after they have a viral illness caused by cytomegalovirus (CMV) or a bacterial infection caused by Helicobacter pylori. Children are not born with this disease, and it is not passed from parents to their children.[2]. The adult form linked with overexpression of transforming growth factor alpha (TGF-α). The adult variety is four times more common in men, primarily affecting men between ages 30 and 60.

The presenting symptoms are

Ménétrier's disease increases a person's risk of stomach cancer.[3]

Microscopically, the disease is characterized by hyperplasia of the crypts, which are elongated and may appear cystic or corkscrew-like. Since it predisposes to stomach cancer, periodic endoscopic surveillance is mandated. CMV-related Ménétrier is often self-limited.

The disease must be diagnosed by x-ray (rare) or endoscopy and biopsy of the stomach. In adults, treatment may include medications to relieve ulcer symptoms and treat inflammation, and a high-protein diet. Part or all of the stomach may need to be removed if the disease is severe. Pediatric cases are normally treated for symptoms with the disease clearing up in weeks to months.

Other forms of hyperplastic gastropathy include Zollinger-Ellison syndrome.

Introduction

Ménétrier's disease is a rare, premalignant disease of the mucous glands of the stomach characterized by massive gastric folds. The most commonly involved location is the fundus and corpus of the stomach.

Historical Perspective

In 1888, Pierre Ménétrier, a French pathologist coined the term Ménétrier's disease after describing mucosal hypertrophy involving part or all of the stomach.

Classification

There is no established system for the classification of Ménétrier's disease.

Pathophysiology

The exact pathogenesis of Ménétrier's disease is not fully understood. However, it is thought that Ménétrier's disease is often due to excessive secretion of transforming growth factor alpha (TGF-α).(PMID 18321437) seen especially in infections such as CMV, H.pylori and herpes simplex.

  • Overproduction of transforming growth factor-α leads to increased signaling of the epidermal growth factor receptor (EGFR), a transmembrane receptor with tyrosine kinase activity.
  • Upon activation, the EGFR activates a series of intracellular signaling pathways that leads to increased cell proliferation.
  • Excessive secretion of TGF-α may lead to gastric foveolar cell (surface mucous cells) hyperplasia, edema, and variable degrees of inflammation.
  • The normal gastric glands such as chief or parietal cells are replaced by mucus-secreting cells leading to decreased effective gastric acid production.
  • An increase in intercellular permeability results in protein loss. In this disorder, tight junctions between cells are wider than those found in healthy subjects, and it is believed that proteins traverse the gastric mucosa through these widened spaces.
  • Hyperplasia of gastric foveolar cells leads to excessive mucus production and enlarged gastric folds.
  • The gastric pits are elongated and tortuous.
  • In addition, there is widening of gap junctions between gastric epithelial cells which may lead to exudation of protein and protein-losing enteropathy.
  • Mucosal hyperplasia and decreased chief and parietal cells leads to protein loss from the stomach, excessive mucus production, and hypochlorhydria or achlorhydria.
  • However, Ménétrier's disease is typically but not always associated with protein-losing gastropathy and hypochlorhydria.

Genetics

  • Genes involved in the pathogenesis of Ménétrier's disease include mutation in SMAD4 associated with juvenile polyposis can lead to a mixed hypertrophic/polypoid gastropathy.
    • A dominant 1244_1247delACAG mutation of SMAD4 was identified in each of the subjects with JPS as well as in each of the subjects with MD. (PMID 27375208)
  • Overproduction of TGF-α leads to overactivation of EGF which may lead to hyperplasia of surface mucous cells.
  • Some researchers suggest a unifying hypothesis which suggest TGF-β–SMAD4 pathway inactivation and TGF-α overexpression related to Hp infection ultimately leads to Ménétrier's disease(PMID 22748914).

Gross pathology

  • Polypoid gastric folds
  • Large cobblestone or cerebriform gastric folds

Microscopic pathology

  • Irregular hypertrophic mucosal folds
  • Mucosa have swollen, spongy appearance subdivided by creases
  • In rare cases, Ménétrier's disease may have hyperplastic gastric polyps
  • Decreased parietal and chief cells with replacement by mucous glands.
  • Intraepithelial lymphocytosis
  • Glandular tortuosity and dilation,
  • Marked reduction in parietal cell number

Associated conditions

  • H.pylori infection
  • CMV gastritis
  • HIV
  • Gastric cancer

Similar conditions

Conditions that may present with enlarged gastric folds other than Ménétrier's disease include Zollinger Ellison syndrome, inflammatory gastritis, gastric adenomas (lymphoma, carcinoma), granulomatous gastritis, gastric varices, and Zollinger Ellison syndrome and hereditary conditions (such as Familial adenomatous polyposis)

Disease Age of onset Risk factors Gastric area involved Type of gastric glands involved Inflammatory cells Symptoms Progression to malignancy
Menetrier's Disease 40-60s H.pylori infection

CMV gastritis

Body & fundus Mucosal cells Lymphocytes Nausea, vomiting, abdominal pain and peripheral edema +
Zollinger Ellison syndrome 50s MEN 1 syndrome Fundus Parietal and mucosal cells Neutrophils Recurrent peptic ulcers at unsual locations -
Inflammatory & hyperplastic polyps 50s Gastritis and H.pylori Antrum Mucosal cells Neutrophils and lymphocytes Nausea, vomiting with epigastric or abdominal pain +/-
Granulomatous gastritis Variable History of prior surgery Body Mucosal cells Neutrophils and lymphocytes Nausea, vomiting with epigastric or abdominal pain -
Familial adenomatous polyposis 50s Mutation in APC gene Body & fundus Parietal cells None None +
Adenomas (gastric) 60s Chronic gastritis and intestinal metaplasia Antrum Dysplastic cells Variable Nausea, vomiting with epigastric or abdominal pain +

Diagnostic Criteria

  • There is no established criteria for the diagnosis of Ménétrier's disease. However, an endoscopic biopsy, chromium-labeled albumin test (GI protein loss), and 24-hour pH monitoring can establish the diagnosis of Ménétrier's disease. Endoscopy shows typical gastric mucosal changes and biopsy establishes the histopathological variant of Ménétrier's disease and also to rule out gastric carcinoma or lymphoma.
  • The most accurate method to diagnose Ménétrier's disease includes testing such as oesophagogastroduodenoscopy with gastric pH, serum albumin and full-thickness mucosal biopsy of the involved gastric mucosa.

History and Symptoms

  • Common symptoms of Ménétrier's disease include epigastric pain, dyspepsia, anorexia, vomiting, weight loss, and edema.
  • Younger age of onset, male predominance

Prognosis

The mean age at diagnosis is 5 yr (range: 2 days-17 yr) Ménétrier's disease usually lasts 2-14 wk, with complete resolution being the rule

Physical Examination

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

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Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].

OR

Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

OR

[Test] is usually normal among patients with [disease name].

OR

Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].

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There are no diagnostic laboratory findings associated with [disease name].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name]. The upper GI series might show thickened gastric folds.

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Medical Therapy

Tthe mainstay of therapy for Ménétrier's disease is supportive care with intravenous albumin (if symptomatic) and parenteral nutritional supplementation. However, some benefit has also been observed with the use of anticholinergic drugs, acid suppression, octreotide and H. pylori eradication.

Surgery

Surgery is not the first-line treatment option for patients with Ménétrier's disease. Surgery (subtotal/total gastrectomy) is usually reserved for patients with either massive protein loss unresponsive to medical therapy or with dysplasia or carcinoma.


References

Additional Resources

  • Rubin's Pathology, Clinicopathological Foundations of Medicine, 4th edition, Rubin, Gorstein, Rubin, Schwarting, Strayer. Lippincott Williams & Wilkins. ISBN 0-7817-4733-3

Cost Effectiveness of Ménétrier's disease

| group5 = Clinical Trials Involving Ménétrier's disease | list5 = Ongoing Trials on Ménétrier's disease at Clinical Trials.govTrial results on Ménétrier's diseaseClinical Trials on Ménétrier's disease at Google


| group6 = Guidelines / Policies / Government Resources (FDA/CDC) Regarding Ménétrier's disease | list6 = US National Guidelines Clearinghouse on Ménétrier's diseaseNICE Guidance on Ménétrier's diseaseNHS PRODIGY GuidanceFDA on Ménétrier's diseaseCDC on Ménétrier's disease


| group7 = Textbook Information on Ménétrier's disease | list7 = Books and Textbook Information on Ménétrier's disease


| group8 = Pharmacology Resources on Ménétrier's disease | list8 = AND (Dose)}} Dosing of Ménétrier's diseaseAND (drug interactions)}} Drug interactions with Ménétrier's diseaseAND (side effects)}} Side effects of Ménétrier's diseaseAND (Allergy)}} Allergic reactions to Ménétrier's diseaseAND (overdose)}} Overdose information on Ménétrier's diseaseAND (carcinogenicity)}} Carcinogenicity information on Ménétrier's diseaseAND (pregnancy)}} Ménétrier's disease in pregnancyAND (pharmacokinetics)}} Pharmacokinetics of Ménétrier's disease


| group9 = Genetics, Pharmacogenomics, and Proteinomics of Ménétrier's disease | list9 = AND (pharmacogenomics)}} Genetics of Ménétrier's diseaseAND (pharmacogenomics)}} Pharmacogenomics of Ménétrier's diseaseAND (proteomics)}} Proteomics of Ménétrier's disease


| group10 = Newstories on Ménétrier's disease | list10 = Ménétrier's disease in the newsBe alerted to news on Ménétrier's diseaseNews trends on Ménétrier's disease


| group11 = Commentary on Ménétrier's disease | list11 = Blogs on Ménétrier's disease

| group12 = Patient Resources on Ménétrier's disease | list12 = Patient resources on Ménétrier's diseaseDiscussion groups on Ménétrier's diseasePatient Handouts on Ménétrier's diseaseDirections to Hospitals Treating Ménétrier's diseaseRisk calculators and risk factors for Ménétrier's disease


| group13 = Healthcare Provider Resources on Ménétrier's disease | list13 = Symptoms of Ménétrier's diseaseCauses & Risk Factors for Ménétrier's diseaseDiagnostic studies for Ménétrier's diseaseTreatment of Ménétrier's disease

| group14 = Continuing Medical Education (CME) Programs on Ménétrier's disease | list14 = CME Programs on Ménétrier's disease

| group15 = International Resources on Ménétrier's disease | list15 = Ménétrier's disease en EspanolMénétrier's disease en Francais

| group16 = Business Resources on Ménétrier's disease | list16 = Ménétrier's disease in the MarketplacePatents on Ménétrier's disease

| group17 = Informatics Resources on Ménétrier's disease | list17 = List of terms related to Ménétrier's disease


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