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1.
Cureus ; 16(2): e55076, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38550419

RESUMO

This is the case of a 52-year-old Indian lady who presented with hematemesis, severe anemia, and an abdominal lump in cardiac failure. On radiographic evaluation, the lesion appeared to be gross circumferential asymmetric proximal gastric wall thickening, with suspicion of gastric lymphoma or tubercular hypertrophic gastritis. After stabilization with multiple transfusions, she underwent proximal D2 gastrectomy with esophago-gastric anastomosis and a total splenectomy. Grossly, the gastric rugae appeared to be hypertrophied and firm. No growth was identified grossly; however, necrotic areas were identified at the distal end. Microscopic examination of multiple sections studied showed significant foveolar hyperplasia, tortuous glands, and a few cystically dilated foveolar glands, which were limited up to the muscle layer. Mild serosal congestion was seen. No atypia or invasion was seen. An impression to consider is the possibility of Ménétrier's disease (MD). MD is an acquired protein-losing enteropathy with giant gastric rugal folds, decreased acid secretion, and increased gastric mucous production. Radiographically, endoscopically, and grossly, the condition can be confused with malignant lymphoma or carcinoma. It is difficult to diagnose, and histopathological confirmation of the resected specimen is needed for a definitive diagnosis. Our intention in presenting this case is to emphasize that MD can present as massive hematemesis and should be considered in a differential diagnosis. Surgical treatment by total or partial gastrectomy is recommended for cases with persistent, debilitating symptoms or a risk of cancer.

2.
Cureus ; 15(9): e45537, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37868436

RESUMO

We present a case report describing the diagnosis and management of a patient who presents with a rare diagnosis of Menetrier's disease. This condition poses a diagnostic challenge to clinicians due to its nonspecific clinical presentation and is oftentimes misdiagnosed for more common gastric disorders. Menetrier's disease is characterized by gastric mucosal hypertrophy and subsequent protein loss, resulting in gastric symptoms and widespread edema. While the etiology remains unclear, notable associations have been observed with Helicobacter pylori (H. pylori) infection and overexpression of transforming growth factor-alpha (TGF-a). The management often involves supportive measures with medical and surgical interventions for refractory cases and when necessary. This report includes a comprehensive review of the literature on the clinical presentation, diagnostic approach, and management of this rare disease. By documenting such cases in the medical literature, we aim to enhance the clinician's ability to recognize and manage this disorder, thereby preventing the development of more severe manifestations such as gastric carcinoma.

3.
World J Gastrointest Endosc ; 15(1): 10-18, 2023 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-36686066

RESUMO

BACKGROUND: Ménétrier's disease is a rare condition characterized by enlarged gastric folds, usually located in the whole body and fundus of the stomach. This report presents an unusual case of localized Ménétrier's disease elevated by a submucosal lipoma and thus looking like a polypoid mass and causing an episode of upper gastrointestinal bleeding. The mass was successfully removed with endoscopic submucosal dissection. CASE SUMMARY: Esophagogastroduodenoscopy was performed on a 76-year-old male patient after an episode of upper gastrointestinal bleeding, manifesting as fatigue and melena. A large polypoid mass (4 cm × 1 cm) with enlarged mucosal folds was found in the body of the stomach, between the lesser curvature and posterior wall. A small ulcer at the distal end of the mass was identified as the source of the bleeding. Biopsy was negative for neoplasia. Computed tomography showed a submucosal lesion beneath the affected mucosa, most likely a lipoma. The mass was removed en bloc with tunneling endoscopic submucosal dissection. Final pathology determined that the mass included Ménétrier's disease and a submucosal lipoma. The patient was scheduled for follow-up esophagogastroduodenoscopy. CONCLUSION: Localized Ménétrier's disease can coexist with a submucosal lipoma creating a polypoid mass with risk of bleeding.

4.
Artigo em Inglês | MEDLINE | ID: mdl-38596548

RESUMO

This review article aims to provide a comprehensive overview of recent epidemiology, pathogenesis, risk factors, and premalignant conditions of gastric cancer. Worldwide, gastric cancer is one of the most common and most fatal cancers. The incidence and mortality remain high in regions such as East Asia and Eastern Europe. Although there is a lower incidence in the United States, it remains a deadly disease. Age, gender, and race are non-modifiable demographic risk factors for developing gastric cancer. There have been several dietary and lifestyle risk factors such as salt preserved foods, N-nitroso compounds containing foods, tobacco smoke, alcohol use, and obesity that have been shown to contribute to the development of gastric cancer. Infections have additionally been shown to have a clear role in the pathogenesis of gastric cancer as Helicobacter pylori eradication has shown a significant reduction in the incidence of gastric cancer as well as other pathogens such as Epstein-Barr virus. There are certain premalignant lesions that increase the risk of developing gastric cancer. These include atrophic gastritis, and intestinal metaplasia amongst others.

5.
Gac. méd. boliv ; 46(2)2023.
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1534498

RESUMO

El síndrome edematoso generalizado o anasarca está presente secundario a un desequilibrio de la homeostasis hídrica, electrolítica y osmolar del organismo, En el adulto el estado de anasarca es más frecuente secundario a insuficiencia cardiaca, en pediatría, el edema que se generaliza está asociado con más frecuencia a bajo aporte proteico, baja síntesis de proteínas o debido a perdida de proteínas de origen gastrointestinal o renal. La disminución de la presión oncótica plasmática genera fuga de líquidos a compartimientos intersticiales de forma generalizada y produce edema. Existen múltiples patologías y mecanismos para la producción del edema generalizado; el conocimiento de la fisiopatología de su desarrollo permite un análisis clínico, de laboratorio y de gabinete que orientan al diagnostico. La infección por citomegalovirus es una causa poco frecuente de edema generalizado, reconocer esta entidad y llegar a un adecuado diagnóstico diferencial es el objetivo de esta revisión.


The generalized edematous syndrome or anasarca is present secondary to an imbalance in the body's water, electrolyte and osmolar homeostasis. In adults, the state of anasarca is more frequent secondary to heart failure; in pediatrics, generalized edema is associated with more frequency due to low protein intake, low protein synthesis or due to protein loss of gastrointestinal or renal origin. The decrease in plasma oncotic pressure generates generalized fluid leakage into interstitial compartments and produces edema. There are multiple pathologies and mechanisms for the production of generalized edema; knowledge of the pathophysiology of its development allows a clinical, laboratory and office analysis that guides the diagnosis. Cytomegalovirus infection is a rare cause of generalized edema; recognizing this entity and reaching an appropriate differential diagnosis is the objective of this review.

6.
Cureus ; 14(10): e30759, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36320788

RESUMO

Ménétrier's disease is a rare, acquired, premalignant disorder of the stomach distinguished by hypertrophic giant mucosal folds in the proximal part of the stomach (body and fundus), diminished acid secretion and excessive mucous production, resulting in hypoalbuminemia. Menetrier's is also known as hypoproteinaemic hypertrophic gastropathy and giant hypertrophic gastritis. We present a case of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier's disease. The etiology of Ménétrier's disease is unknown but it has been linked to raised TGF-α (transforming growth factor alpha protein) in the gastric mucosa causing increased signalling of epidermal growth factor. It is also associated with infections such as cytomegalovirus and Helicobacter pylori. Despite these associations, there have been cases without any of these viruses detected.  Diagnosis is usually made by barium swallow or endoscopic evaluation, which characteristically shows thickened gastric folds, while the biopsies' hallmark feature is massive foveolar hyperplasia. We present a case series of four siblings who presented between 2010 and 2017 with symptomatic iron deficiency anaemia or upper gastrointestinal symptoms with confirmed Ménétrier's disease.

7.
Int J Surg Case Rep ; 98: 107539, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36027830

RESUMO

INTRODUCTION AND IMPORTANCE: Menetrier's disease is a rare type of hypertrophic gastropathy characterized by the atrophy of the gastric parietal cells and dilatation of mucus releasing glands. Hereby, we present a morbid obese patient who has undergone laparoscopic sleeve gastrectomy (LSG) and he has also diagnosed with Menetrier's disease. CASE PRESENTATION: A 67-year-old male patient whose body mass index (BMI) was 39 kg/m2. Preoperative endoscopy was done. There were no pathologies except increased gastric mucosal folds. LSG was done. During the surgery it was noticed that gastric tissue was abnormally thick. After LSG completed, it was observed that there was an abnormal bleeding from the staple line. The staple line was oversewed with 3.0 V-Loc™ and bleeding was stopped. Pathology report was compatible with menetrier's disease. CLINICAL DISCUSSION: Hypoalbuminemia and H. pylori take an important place in diagnosis of Menetrier's disease, but H. pylori was not detected and albumin level was normal in our patient. For certain diagnosis full-thickness gastric biopsy is needed. The routine use of preoperative endoscopy in patients scheduled for bariatric surgery was still controversial until recently. CONCLUSION: This is the first case with menetrier's disease that has undergone LSG. Preoperative endoscopic evaluation before bariatric surgery is crucial. As in this case, it will be effective in terms performing additional intraoperative precautions when necessary and preventing possible complications.

8.
Cureus ; 14(3): e23389, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35475041

RESUMO

There is a wide differential diagnosis within polyposis syndromes. Our case represents an interesting and diagnostically challenging diagnosis involving a 41-year-old male who presented with an incidental gastric mass on imaging and a colonic mass seen on colonoscopy. Following multiple endoscopic evaluations, histological analysis, and genetic testing, the patient was ultimately diagnosed with juvenile polyposis syndrome (JPS)/hereditary hemorrhagic telangiectasia (HHT) despite the initial suspicion for Ménétrier's disease. His disease course was complicated by an acute upper extremity thrombus and diagnosis of colorectal carcinoma. This case highlights the importance of a thorough evaluation when polyposis syndromes are suspected. Prompt and accurate diagnosis can aid in the treatment, surveillance, and prevention of colorectal carcinoma.

9.
World J Gastroenterol ; 28(5): 594-601, 2022 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-35316958

RESUMO

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.


Assuntos
Ressecção Endoscópica de Mucosa , Gastrite Hipertrófica , Helicobacter pylori , Neoplasias Gástricas , Adulto , Feminino , Gastrite Hipertrófica/diagnóstico , Gastrite Hipertrófica/diagnóstico por imagem , Gastroscopia , Humanos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Neoplasias Gástricas/patologia
10.
Mol Clin Oncol ; 15(5): 233, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34650800

RESUMO

Intracholecystic papillary neoplasm of the gallbladder (ICPN) is a type of intraductal papillary neoplasm of the bile duct that occurs in the gallbladder, and is a relatively newer concept. Therefore, there are few reports regarding ICPN. Menetrier's disease is a rare disease characterized by giant hypertrophy of the gastric folds that causes protein-losing gastroenteropathy (PLG). Although Menetrier's disease is a known risk factor for gastric adenocarcinoma, the association between Menetrier's disease and malignancy other than a malignancy of the stomach is unclear. A 69-year-old man presented to the Hokkaido Social Work Association Obihiro Hospital with gallbladder tumours diagnosed by ultrasonography at a previous institution. In addition, he had previously been diagnosed with PLG due to Menetrier's disease. Abdominal contrast-enhanced computed tomography (CT) revealed an irregular mass with a contrast effect at the fundus of the gallbladder on the free abdominal cavity side. Positron emission tomography-CT showed a tumour with a standard uptake value (SUV) of 8.28 at the fundus of the gallbladder. Cholecystectomy and resection of the gallbladder bed were performed. Based on the microscopy findings, the patient was diagnosed with ICPN. Although he had postoperative ileus, he was discharged 14 days postoperatively due to improvement through conservative treatment. Such cases of ICPN complicated with Menetrier's disease are extremely rare. However, patients with Menetrier's disease may need to be screened for malignancies.

11.
World J Clin Cases ; 9(23): 6943-6949, 2021 Aug 16.
Artigo em Inglês | MEDLINE | ID: mdl-34447846

RESUMO

BACKGROUND: Gastric mucosal hypertrophy, also known as Menetrier's disease (MD), is more common in men over 50 years of age, and the cause is unknown. The symptoms of the disease are atypical, mostly accompanied by hypoproteinemia and edema, and sometimes accompanied by symptoms such as epigastric pain, weight loss, and diarrhea. Most experts believe that the site of the disease is mainly located in the fundus of the stomach and the body of the stomach. We found that the site of the disease in this patient involved the antrum of the stomach. CASE SUMMARY: We introduced the case of a 24-year-old woman who had repeated vomiting for 5 d and was admitted to our hospital. After various examinations such as computed tomography and pathology in our hospital, the final diagnosis of the presented case is MD. The salient feature is that the mucosal folds in the fundus and body of the stomach are huge and present in the shape of gyrus. The greater curvature is more prominent, and there are multiple erosions or ulcers on the folds. The patient did not undergo gastric surgery and did not undergo re-examination. She is drinking Chinese medicine for treatment, and her vomiting and abdominal pain symptoms have improved. This disease is relatively rare in clinical practice, and it is easy to be misdiagnosed as gastric cancer, chronic gastritis and gastric lymphoma, etc. CONCLUSION: MD can occur in the antrum, it is necessary to raise awareness of the disease and reduce misdiagnosis.

12.
Medicina (B Aires) ; 81(3): 470-473, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34137712

RESUMO

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.


Assuntos
Gastrite Hipertrófica , Adulto , Anticorpos Monoclonais , Biópsia , Mucosa Gástrica , Gastrite Hipertrófica/diagnóstico , Gastrite Hipertrófica/tratamento farmacológico , Gastroscopia , Humanos , Masculino
13.
Medicina (B.Aires) ; 81(3): 470-473, jun. 2021. graf
Artigo em Inglês | LILACS | ID: biblio-1346488

RESUMO

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.


Assuntos
Humanos , Masculino , Adulto , Gastrite Hipertrófica/diagnóstico , Gastrite Hipertrófica/tratamento farmacológico , Biópsia , Gastroscopia , Mucosa Gástrica , Anticorpos Monoclonais
15.
BMC Vet Res ; 17(1): 98, 2021 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-33653341

RESUMO

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.


Assuntos
Doenças do Cão/patologia , Gastrite Hipertrófica/veterinária , Infecções por Helicobacter/veterinária , Leishmaniose/veterinária , Animais , Doenças do Cão/diagnóstico , Doenças do Cão/microbiologia , Doenças do Cão/parasitologia , Cães , Feminino , Gastrite Hipertrófica/diagnóstico , Gastrite Hipertrófica/tratamento farmacológico , Helicobacter , Infecções por Helicobacter/tratamento farmacológico , Hipoalbuminemia/veterinária , Leishmania/imunologia , Leishmaniose/tratamento farmacológico , Estômago/patologia , Estômago/cirurgia , Vômito/tratamento farmacológico , Vômito/veterinária
16.
Pediatr Gastroenterol Hepatol Nutr ; 24(1): 109-117, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33505900

RESUMO

PURPOSE: Ménétrier disease (MD) was first described in 1888, and 50 cases have been reported until now. We aimed to discuss the etiology, diagnostics, and management of MD in children. METHODS: We searched for case reports published from 2014 till 2019 in English using PubMed. Articles were selected using subject headings and key words of interest to the topic. Interesting references of the included articles were also included. RESULTS: The pathophysiology of MD is still uncertain. However, overexpression of transforming growth factor alpha with transformation of the gastric mucosa has been observed, which may be mediated by genetics and provoked by an infectious trigger. Clinically, MD is diagnosed by abdominal pain, vomiting, anorexia, and edema secondary to hypoalbuminemia. A gastroscopy with biopsy is the gold standard for the diagnosis of MD. In children, the disease is self-limiting and only requires supportive treatment. In general, children have a good prognosis and recover spontaneously within a few weeks. CONCLUSION: Few pediatric cases of MD have been described in recent years, and with all different etiology. Endoscopy with biopsy remains the golden standard for the diagnosis of MD, and in children, the disease is self-limiting.

17.
Case Rep Gastroenterol ; 15(3): 985-993, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-35110986

RESUMO

To advance the diagnostic accuracy of juvenile polyposis syndrome, an important yet often difficult diagnosis, we describe in detail a new and medically significant presentation. This hereditary and high-risk GI cancer syndrome is often associated with hereditary hemorrhagic telangiectasia, as in this 47-year-old female patient with a SMAD4 germline pathogenic mutation. Total gastrectomy revealed giant gastric folds with inflamed foveolar hyperplasia consuming most of the gastric cardia and body but sparing the antrum. Together, this gross and histologic pathology mimics Ménétrier's disease, an exceedingly rare and acquired protein-losing hypertrophic gastropathy. Classical gastric juvenile polyposis almost always and principally involves the antrum with multiple distinctive inflammatory polyps rather than the newly illustrated giant gastric folds of this case. No reports of giant gastric folds in juvenile polyposis have appeared in the literature. The distinction between juvenile polyposis and Ménétrier's disease is essential due to their disparate clinical outcomes and management. The differential considerations for giant gastric folds and inflamed gastric foveolar hyperplasia are fully reviewed. On the basis of this report, the differential for giant gastric folds must now expand to include juvenile polyposis syndrome. Genetic testing for pathogenic germline mutations of the 2 known causative genes of this syndrome, namely SMAD4 and BMPR1A, are readily available and should become part of the evaluation of giant gastric folds, particularly in view of the neoplastic and hereditary aspects of juvenile polyposis syndrome.

18.
J Vet Diagn Invest ; 33(1): 112-115, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33090082

RESUMO

A 14-y-old spayed female Labrador Retriever was presented with an 8-mo history of chronic vomiting. Abdominal ultrasound and gastrointestinal endoscopy revealed a mass protruding into the gastric lumen, with cytologic features suggestive of sarcoma. A partial gastrectomy was performed; the gastric body and antrum were thickened, with a cerebriform appearance of the mucosal surface. Histologic examination revealed a submucosal neoplastic proliferation of fusiform cells variably arranged in irregular bundles and scattered whorls. Fusiform cells strongly reacted to antibodies against vimentin, S100, and neuron-specific enolase; glial fibrillary acidic protein was moderately and multifocally expressed. Pancytokeratin, KIT, α-smooth muscle actin, and desmin were nonreactive. Histologic and immunohistochemical findings suggested a diagnosis of gastric sarcoma with features referable to a non-GIST (gastrointestinal stromal tumor), non-smooth muscle NIMT (non-angiogenic, non-lymphogenic intestinal mesenchymal tumor). The overlying gastric mucosa was thickened by elongated and dilated gastric glands, predominantly lined by intensely periodic acid-Schiff-stained mucous cells. This altered mucosal architecture was suggestive of Ménétrier-like disease. Although this disease has been hypothesized to predispose to gastric adenocarcinoma in dogs, an association with gastric sarcoma has not been documented previously in the veterinary literature, to our knowledge.


Assuntos
Doenças do Cão/patologia , Sarcoma/veterinária , Gastropatias/veterinária , Animais , Cães , Evolução Fatal , Feminino , Embolia Pulmonar/etiologia , Sarcoma/complicações , Sarcoma/patologia , Gastropatias/complicações , Gastropatias/patologia , Neoplasias Gástricas/complicações , Neoplasias Gástricas/patologia , Neoplasias Gástricas/veterinária
19.
Eur J Pediatr ; 180(3): 679-688, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32803424

RESUMO

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.


Assuntos
Infecções por Citomegalovirus , Gastrite Hipertrófica , Criança , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/epidemiologia , Mucosa Gástrica , Gastrite Hipertrófica/complicações , Gastrite Hipertrófica/diagnóstico , Gastrite Hipertrófica/epidemiologia , Humanos , Masculino , Estudos Retrospectivos
20.
Cureus ; 12(11): e11515, 2020 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-33354459

RESUMO

Menetrier's disease (MD) is a rare disease characterized macroscopically by gastric rugae thickening and microscopically by foveolar hyperplasia with glandular atrophy, resulting in luminal protein loss. Different treatment strategies, including antibiotics, prednisone, octreotide, and monoclonal antibodies, have yielded varying degrees of success. Here, we present a rare complication of MD with a gastric outlet obstruction from a large adenoma. However, prior to this complication, dramatic clinical and laboratory improvements were observed after 12 months of treatment with subcutaneous octreotide. We also present a review of the literature for the role of octreotide in the treatment of MD.

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