[Menetrier's disease and its association with gastric hyperplastic polyps]. / Enfermedad de Menetrier y su asociación a pólipos gástricos hiperplásicos.

Menetrier's disease represents a low prevalence clinical entity, characterized by complexity in its diagnosis, particularly due to the need to exclude its potential association with gastric cancer. In this context, we present the clinical case of a 54-year-old male with nonspecific gastrointestinal symptoms and hypoalbuminemia. During the upper endoscopy procedure, a noticeable thickening of gastric folds was observed, associated with multiple polypoid lesions in the stomach, predominantly in the fundus and body. Since the patient did not show improvement in symptoms and given the inability to rule out gastric cancer, total gastrectomy was chosen as the treatment. Surgical specimen and histology confirmed the presence of Menetrier's disease.

Curative resection with endoscopic submucosal dissection of early gastric cancer in Helicobacter pylori-negative Ménétrier's disease: A case report.

BACKGROUND: Adult-onset Ménétrier's disease is strongly associated with Helicobacter pylori (H. pylori) infection and an elevated risk of carcinogenesis. Cases of early-stage gastric cancer developed in H. pylori-negative Ménétrier's disease are extremely rare. We report a case of early gastric cancer in H. pylori-negative Ménétrier's disease that was curatively resected with endoscopic submucosal dissection (ESD). CASE SUMMARY: A 60-year-old woman was referred to our hospital after her medical examination detected anemia. Contrast-enhanced upper gastrointestinal (UGI) radiography revealed translucency of the nodule-aggregating surface with giant rugae. Blood tests showed hypoproteinemia and were negative for serum H. pylori immunoglobulin G antibodies. The 99mTc-DTPA-human serum albumin scintigraphy showed protein loss from the stomach. UGI endoscopy showed a 40-mm protruding erythematous lesion on giant rugae of the greater curvature of lower gastric body, suggesting early-stage gastric cancer due to Ménétrier's disease. En bloc resection with ESD was performed for diagnosis and treatment. Histology of ESD showed well-differentiated tubular adenocarcinoma. The cancer was confined to the mucosa, and complete curative resection was achieved. Foveolar hyperplasia and atrophy of the gastric glands were observed in non-tumor areas, histologically corresponding to Ménétrier's disease. Three years after ESD, gastric cancer had not recurred, and Ménétrier's disease remained in remission with spontaneous regression of giant gastric rugae. CONCLUSION: Complete curative resection was achieved through ESD in a patient with early-stage gastric cancer and H. pylori-negative Ménétrier's disease.

Menetrier's disease. A diagnostic and therapeutic challenge.

We present a rare case of hypertrophic gastropathy associated with protein loss. A 35-year-old man was hospitalized for bowel habit changes, abdominal pain, generalized edema and symptomatic anemia. Pertinent laboratory findings included iron deficiency anemia (Hb 6.7g/dl, ferritin 5 ng/ml) and marked hypoalbuminemia (albumin 2.5 g/dl). Endoscopic biopsy samples of giant gastric folds observed along the greater gastric curvature revealed foveolar hyperplasia and significant parietal cell loss. Endoscopic ultrasonography showed gastric parietal thickening with preserved architecture and normal gastric wall layers. Menetrier disease was diagnosed and the patient treated with cetuximab, a monoclonal antibody that inhibits ligand binding of transforming growth factor alpha (TGFa), preventing gastric mucosa cell proliferation. After twelve months of treatment, the patient referred symptoms improvement, and gastric biopsy levels of the proliferation marker protein Ki-67 had decreased.

Presentamos un caso infrecuente de gastropatía hipertrófica asociada a pérdida de proteínas. Un hombre de 35 años fue hospitalizado por cambios en los hábitos intestinales, dolor abdominal, edema generalizado y anemia sintomática. Los hallazgos de laboratorio pertinentes incluyeron anemia ferropénica (Hb 6.7 g/dl, ferritina 5 ng/ml) e hipoalbuminemia marcada (albúmina 2.5 g/dl). Las muestras de biopsia endoscópica de pliegues gástricos gigantes observados a lo largo de la curvatura mayor gástrica revelaron hiperplasia foveolar y pérdida significativa de células parietales. La ecografía endoscópica mostró engrosamiento parietal gástrico con arquitectura conservada y capas de pared gástrica normales. Se diagnosticó enfermedad de Menetrier y se trató al paciente con cetuximab, un anticuerpo monoclonal que inhibe la unión del ligando del factor de crecimiento transformante alfa (TGFa), evitando la proliferación de células de la mucosa gástrica. Después de doce meses de tratamiento, el paciente refirió mejoría de los síntomas y los niveles de la proteína marcadora de proliferación Ki-67 en biopsia gástrica habían disminuido.

Menetrier's disease. A diagnostic and therapeutic challenge / Enfermedad de Menetrier. Un desafío diagnóstico y terapéutico

Abstract We present a rare case of hypertrophic gastropathy associated with protein loss. A 35-year-old man was hospitalized for bowel habit changes, abdominal pain, generalized edema and symptomatic anemia. Pertinent laboratory findings included iron deficiency anemia (Hb 6.7g/dl, ferritin 5 ng/ml) and marked hypoalbuminemia (albumin 2.5 g/dl). Endoscopic biopsy samples of giant gastric folds observed along the greater gastric curvature revealed foveolar hyperplasia and significant parietal cell loss. Endoscopic ultrasonography showed gastric parietal thickening with preserved architecture and normal gastric wall layers. Menetrier disease was diagnosed and the patient treated with cetuximab, a monoclonal antibody that inhibits ligand binding of trans forming growth factor alpha (TGFa), preventing gastric mucosa cell proliferation. After twelve months of treatment, the patient referred symptoms improvement, and gastric biopsy levels of the proliferation marker protein Ki-67 had decreased.

Resumen Presentamos un caso infrecuente de gastropatía hipertrófica asociada a pérdida de proteínas. Un hombre de 35 años fue hos pitalizado por cambios en los hábitos intestinales, dolor abdominal, edema generalizado y anemia sintomática. Los hallazgos de laboratorio pertinentes incluyeron anemia ferropénica (Hb 6.7 g/dl, ferritina 5 ng/ml) e hipoal buminemia marcada (albúmina 2.5 g/dl). Las muestras de biopsia endoscópica de pliegues gástricos gigantes observados a lo largo de la curvatura mayor gástrica revelaron hiperplasia foveolar y pérdida significativa de células parietales. La ecografía endoscópica mostró engrosamiento parietal gástrico con arquitectura conservada y capas de pared gástrica normales. Se diagnosticó enfermedad de Menetrier y se trató al paciente con cetuximab, un anticuerpo monoclonal que inhibe la unión del ligando del factor de crecimiento transformante alfa (TGFa), evitando la proliferación de células de la mucosa gástrica. Después de doce meses de tratamiento, el paciente refirió mejoría de los síntomas y los niveles de la proteína marcadora de proliferación Ki-67 en biopsia gástrica habían disminuido.

Ménétrier-like disease in a Pointer with concurrent granulomatous gastritis, helicobacteriosis and leishmaniosis: a case report.

BACKGROUND: Ménétrier-like disease is a rare hypertrophic canine gastropathy, reported in only seven dogs. Clinical signs are vomiting, anorexia and weight loss. Macroscopically, giant cerebriform gastric mucosal folds are typically seen in the corpus and fundus of the stomach. Histopathologically, fundic mucous cell hyperplasia and loss of parietal and chief cells are typical. CASE PRESENTATION: A nine-year-old spayed female Pointer had a history of intermittent vomiting, marked weight loss and hypoalbuminaemia. A gastroduodenoscopy was performed three times within three months with macroscopic changes remaining the same. The gastric mucosa of the corpus, fundus and proximal antrum was markedly irregular, with cerebriform mucosal folds. In the first gastric biopsies, histopathology revealed a moderate granulomatous gastritis, with a severe manifestation of Helicobacter-like organisms. Treatment for Helicobacter spp. decreased the vomiting slightly. The dog was diagnosed with concurrent leishmaniosis; the conventional anti-Leishmania treatment decreased the vomiting moderately, the hypoalbuminaemia resolved and the dog gained weight back to a normal body condition. Granulomatous gastritis was not present in the gastric biopsies after these treatments. The dog increased vomiting when palliative treatment (maropitant citrate, ondansetron and esomeprazole) was discontinued, and thus, full-thickness biopsies of the stomach were taken and Ménétrier-like disease was diagnosed. The affected area was too large to be surgically removed; thus, palliative treatment was reinstated. The dog remained clinically well 39 months after the first clinical presentation. CONCLUSIONS: This is the first report of Ménétrier-like disease in a dog with a simultaneous manifestation of granulomatous gastritis, helicobacteriosis and leishmaniosis. The clinical signs decreased after treatment of helicobacteriosis and leishmaniosis, but vomiting remained probably as a sign of Ménétrier-like disease. Treatment options for dogs are surgical removal of the abnormal area or palliative treatment. In humans, promising results for a cure have been shown with cetuximab treatment, a human monoclonal antibody, but no canine antibody is commercially available yet. The dog here was doing well 39 months after first presentation, which is the longest reported survival time for Ménétrier-like disease with only palliative treatment in dogs. Full-thickness biopsies are preferred in macroscopic hypertrophic lesions of the stomach for better assessment of Ménétrier-like disease.

Menetrier disease and Cytomegalovirus infection in paediatric age: report of three cases and a review of the literature.

Paediatric Menetrier disease (PMD) is a protein-losing gastropathy, presenting with generalized oedema and abdominal symptoms. PMD commonly has an acute course and may be associated with Cytomegalovirus (CMV) infection. The aim of this retrospective study is to assess the epidemiological and clinical data, diagnostic procedures, treatment and outcome of CMV-associated PMD. The medical charts of the patients with PMD and CMV infection diagnosed at our hospital have been reviewed. Then, a systematic literature's review of all the cases of PMD and a selection of those associated with CMV infection have been performed. Three previously healthy boys were admitted for vomiting and oedema. Endoscopy showed hypertrophic gastric folds and CMV infection was diagnosed. Albumin was administered in all cases, with clinical resolution within few weeks. In literature, PMD has been described in 150 children and the association between CMV and PMD was found in 89 cases. Clinical and laboratory data, radiological and histological exams, therapy and outcome were reviewed.Conclusions: Basing on the present experience and on the current knowledge, PMD has a benign course without long-term sequelae. Although PMD is rare in children, we recommend paediatricians to consider CMV-related PMD when facing children with vomiting and diffuse oedema. What is Known: • Paediatricians should consider Menetrier disease (MD) when facing oedematous child complaining of abdominal symptoms with hypoalbuminemia, without proteinuria and liver dysfunction. • Typical ultrasound features (hypertrophic gastric folds) suggest such condition which requires endoscopy and biopsy for definitive diagnosis. What is New: • A familial susceptibility to CMV gastric infection has been recently suggested; thus when suspecting MD, the family history of gastric diseases should be investigated. • Menetrier disease has been found associated with other unusual conditions either benign (such as gastric bezoar) or malign as neoplasms (acute lymphatic leukaemia and adenocarcinoma) even in children.

Enfermedad de Ménétrier. Presentación de un caso y revisión de la literatura / Ménétrier's disease. Case report and literature review / Doença de Ménétrier. Apresentação de caso e revisão de literatura

RESUMEN Se presentó el caso clínico de un adulto de 56 años de edad, atendido en el Servicio de Cirugía del Hospital Clínico Quirúrgico Docente "Dr. Ambrosio Grillo", provincia Santiago de Cuba, que fue operado de urgencia con el diagnóstico preoperatorio de cáncer gástrico complicado. El estudio anatomopatológico no confirmó dicho diagnóstico y sí evidenció enfermedad de Ménétrier. El paciente no presentó complicaciones en su evolución posoperatoria, por lo que se egresó 7 días después.

ABSTRACT A clinical case of a 56-year-old adult, was treated at the Surgery Service of the Teaching Surgical Clinical Hospital "Dr. Ambrosio Grillo", Santiago de Cuba province, which was operated urgently with the preoperative diagnosis of complicated gastric cancer. The pathological study did not confirm this diagnosis and did show Ménétrier disease. The patient did not present complications in his postoperative evolution, so he discharged the hospital 7 days later.

RESUMO O caso clínico de um adulto de 56 anos, atendido no Serviço de Cirurgia do Hospital Clínico Cirúrgico de Ensino "Dr. Ambrosio Grillo", província de Santiago de Cuba, operada com urgência com o diagnóstico pré-operatório de câncer gástrico complicado. O estudo patológico não confirmou esse diagnóstico e mostrou a doença de Ménétrier. O paciente não apresentou complicações em sua evolução pós-operatória e, assim, aos 7 dias ele saui do hospital.

Unusual case of adult familial Menetrier disease in siblings.

Menetrier disease is a rare disease characterised by hyperplasia of the gastric epithelium and large gastric folds. We present a case of a 58-year-old woman who was referred with iron deficiency anaemia, with a family history of a sibling who had undergone gastrectomy for presumed gastric malignancy. Endoscopy showed prominent gastric mucosal folds and biopsies showed hyperplastic gastric mucosa, with prominent foveolar hyperplasia suggestive of Menetrier disease. Further information about her brother's diagnosis was sought, and it was found that his pathology after gastrectomy showed diffuse glandular hyperplasia also in keeping with Menetrier disease. Adult familial Menetrier disease has so far been a rarity in the literature-review elicits five previous cases of this presentation in siblings.

Ménétrier's disease : a case of successful treatment using long-acting octreotide.

The authors describe a 31 years old male, admitted for hematemesis, epigastric pain and lower limb edema. Laboratorial data showed haemoglobin 18.4g/dl, total proteins 2.8g/dl, albumin 1.6g/dl and hipogammaglobulinaemia. 24h urinary proteins were normal. HIV and CMV serology were negative. Upper GI endoscopy revealed markedly enlarged gastric folds covered by abundant exudative fluid. Endoscopic ultrasound showed ascites, pleural effusion and gastric wall thickening with mucosa expansion and intact submucosa. In gastric biopsies foveolar hyperplastic and regenerative mucosa were observed being suggestive of Ménétrier´s disease. Helicobacter pylori was not detected. Albumin replacement and diuretics corrected anasarca and long-acting octreotide was instituted. Nine months later, the patient was asymptomatic, serum proteins were normal (albumin 4.6g/dl and total proteins 6.5g/dl), signs of endoscopic improvement were observed with marked reduction in gastric folds and mucosal inflammation and no ultrastructural changes were detected in gastric specimens sent for electron microscopy. Ménétrier´s Disease (MD) is a rare form of hypertrophic gastropathy characterized by massive enlargement of gastric folds causing marked protein exudation. The increase in tight junction diameter is the most consistent ultraestrutural change. Octreotide is a somatostatin analogue that acts by modulating TGFαEGFR pathway, which has been associated with the pathogenic mechanisms. As well as other cases reported in literature, this case report highlights the potential of long-acting octreotide for MD treatment avoiding more expensive therapies like cetuximab and gastrectomy.

Protein-losing gastropathy associated with cytomegalovirus in two sisters - Case reports and review of the literature.

Ménétrier's disease is a protein-losing gastropathy that is uncommon in childhood. Its symptoms are unspecific, with abdominal pain, vomiting, and edema. Blood tests show hypoproteinemia and hypoalbuminemia, and upper digestive endoscopy reveals giant gastric folds. In children, cytomegalovirus has been identified as a possible cause. Here we describe two sisters presenting with Ménétrier's disease, 2 years apart. This diagnosis should be considered in the presence of hypoalbuminemia in children when a nephrotic syndrome is excluded.

Ménétrier's disease presenting as recurrent unprovoked venous thrombosis: a case report.

BACKGROUND: Acquired thrombophilia is a potential sequela of malignancy, chronic inflammation, and conditions characterized by severe protein deficiency (for example, nephrotic syndrome, protein-losing enteropathy). As such, venous thrombosis is often a feature, and occasionally a presenting sign, of systemic disease. Ménétrier's disease is a rare hyperplastic gastropathy that may lead to gastrointestinal protein loss and hypoalbuminemia. To date, reports of venous thrombosis associated with Ménétrier's disease are exceedingly scarce. CASE PRESENTATION: We report the case of a 40-year-old white man who presented with unprovoked deep venous thrombosis, pulmonary embolism, and renal vein thrombosis. Upon receiving therapeutic anticoagulation, he developed severe gastrointestinal bleeding, and endoscopic evaluation led to a diagnosis of Ménétrier's disease. A laboratory workup revealed deficiency of protein C, protein S, and antithrombin III, as well as markedly elevated levels of factor VIII. He was determined to have an acquired thrombophilia as a direct result of Ménétrier's disease. CONCLUSIONS: This case describes an acquired thrombophilic state in a patient with Ménétrier's disease and profound hypoalbuminemia. Although this association is rarely described, we discuss the probable mechanisms leading to our patient's thrombosis. Specifically, we posit that his gastrointestinal protein loss led to a deficiency of several anticoagulant proteins and a compensatory elevation in factor VIII, as occurs in nephrotic syndrome and inflammatory bowel disease. Of note, this patient's recurrent venous thrombosis was the initial clinical sign of his gastrointestinal pathology.

Enfermedad de Menetrier como entidad poco común / Ménétrier's disease as a rare entity

La enfermedad de Menetrier se define como la poliposis gástrica múltiple. Es una entidad infrecuente y se caracteriza por pólipos hiperplásicos de menos de 2 centímetros de diámetro, que rara vez se malignizan. Se presenta una paciente con enfermedad de Menetrier, que llevó seguimiento endoscópico durante tres años y debido al aumento progresivo del número de pólipos y la sintomatología se realizó una gastrectomía subtotal sin complicaciones intra ni posoperatorias(AU)

The Ménétrier's disease is defined as multiple gastric polyposis. It is an infrequent entity characterized by hyperplastic polyps less than 2 centimeters in diameter, which rarely become malignant. We present a patient with Ménétrier's disease, who underwent endoscopic follow-up for three years and, due to the progressive increase in the number of polyps and the symptomatology, was performed a subtotal gastrectomy without intraoperative or postoperative complications(AU)

Ménétrier disease manifested by polyposis and involved in both the small bowel and entire colon: A Case Report.

INTRODUCTION: Ménétrier disease (MD) is rare that is involved in both the small bowel and entire colon. THE MAIN SYMPTOMS AND THE IMPORTANT CLINICAL FINDINGS: We describe a case of a 76-year-old male patient whose clinical presentations include intermittent diarrhea, epigastric pain, nausea, vomiting, asitia, and weight loss. An endoscopy was performed showing a large number of irregular forms and different sizes of polypoid lesions in the gastrointestinal tract, which is rare for MD. THE MAIN DIAGNOSES, THERAPEUTICS INTERVENTIONS, AND OUTCOMES: Herein, this case was diagnosed as MD, mainly dependent on endoscopic evaluation, typical clinical symptoms, and histopathological examination of biopsy. As this patient was also infected with Helicobacter pylori, the eradication of H pylori was administered. Meanwhile, a high-protein diet was enjoined, the aforementioned patient's symptoms were alleviated evidently after 1 month. CONCLUSION: Although the etiology of MD remained undetermined, we showed that eradication of H pylori in this case might contribute to the disease remission. This study enlarged the present understanding of MD.

[An infrequent cause of iron-deficiency anemia: Ménétrier disease]. / Una causa infrecuente de anemia ferropénica: Enfermedad de Ménétrier.

Ménétrier disease is a rare condition of an unknown origin, with a wide spectrum of clinical signs and symptoms at presentation. Diagnosis is made through the combination of clinical, analytical, endoscopic and histological data. Surgery is the classical treatment. However, new medical approaches have emerged.

An unusual presentation of Ménétrier's Disease.

Ménétrier's Disease (MD) is a rare acquired hypertrophic gastropathy characterized by giant hypertrophic rugal folds, hypochlorhydria, and hypoproteinemia. The definitive etiology of MD is controversial, although infection with Helicobacter pylori (H. pylori) has been implicated in adults. It presents as a constellation of symptoms including epigastric pain, fatigue, vomiting, weight loss, anorexia, and edema. None of these signs and symptoms is specific for the disease. The gastrointestinal symptoms and the degree of hypoalbuminemia can be profound, the latter resulting from the leakage of protein from the gastric lining. The disease is more common in males. Herein, we report a case of a young woman presenting with the chief complaint of peripheral edema with minimal gastrointestinal symptoms, which was diagnosed as MD on endoscopic evaluation and histopathological examination of gastric biopsy. A high index of suspicion is needed to correctly diagnose this condition for its optimal management.

Disease recurrence following cetuximab completion and declining a gastrectomy: what next to manage Ménétriers disease?

Ménétriers disease is a rare mucosal hyperproliferative disorder of the stomach, however, the evidence for long-term care remains limited, especially if a gastrectomy is declined. We present a case of 25-year-old Caucasian woman with a history of end-stage renal failure (ESRF) who experienced worsening symptoms of abdominal pain, haematemesis and abdominal swelling, with her serum albumin dropping to 20 g/L and haemoglobin to 4.9 g/dL. Endoscopy showed markedly hyperplastic gastric folds consistent with Ménétriers disease, confirmed histologically by gland dilation and gastric pit expansion. Intravenous cetuximab was prescribed for 12 months, with clinical, biochemical and endoscopic improvement. However, 5 weeks post cetuximab completion, there was relapse to 50% gastric coverage with Ménétriers. A discussion around gastrectomy was rejected by the patient. This is the first report of relapsing Ménétriers disease in a female patient with ESRF; we suggest that long-term cetuximab should be considered if a gastrectomy is declined.