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Answer to the image of the month question (page 968): Menetrier disease with premalignant transformation
October 2002
contacting the ABP. Additional information including eligibility requirements may also be found on the ABP website. Each applicant will be considered individually and must be acceptable to the ABP.
NOTICES
1419
Contact: American Board of Pediatrics, 111 Silver Cedar Court, Chapel Hill, North Carolina 27514-1513; call (919) 929-0461; fax (919) 918-7114 or (919) 929-9255; or visit the website at www. abp.org.
Answer to the Image of the Month Question (page 968): Menetrier Disease With Premalignant Transformation Upper gastrointestinal endoscopy showed large thickened rugal folds projecting into the gastric lumen (Figure A). There were multiple associated large polypoid lesions distributed throughout the body, fundus, and cardia of stomach. There was characteristic, relative sparing of the gastric antrum. Abdominal computerized tomography (CT) demonstrated diffuse thickening of the gastric wall in the form of rugal fold enlargement (Figure B). A maximum wall thickness of 4 cm was measured. The disease distribution noted on endoscopy correlated well with CT findings. The gastric lumen appeared diminutive and filled with giant rugal folds. Small bowel wall-thickening was seen in keeping with the typical protein-losing enteropathy and the patient’s known hypoalbuminemic status. Gastric biopsy demonstrated extensive foveolar hyperplasia with multifocal high-grade dysplasia. No definite carcinoma was seen. The patient had a total gastrectomy to eliminate the real risk of progression to gastric carcinoma and to prevent further protein loss. The pathological diagnosis was Menetrier disease with multifocal high-grade dysplasia. Menetrier disease (hypertrophic gastropathy) is a rare, idiopathic condition, seen most commonly in the fourth to sixth decade of life, and is characterized by hyperplasia of gastric mucosal epithelium. There is characteristic mucus hypersecretion leading to a negative nitrogen balance and hypoproteinemic state, and frequently, there is hyposecretion of acid. The incidence of progression to gastric carcinoma is estimated at 2%–15%. In patients not responding to medical therapy, total gastrectomy should be considered. The differential diagnosis for the endoscopic and CT appearances includes lymphoma, Zollinger–Ellison syndrome, amyloidosis, eosinophilic gastritis, and infiltrative neoplastic entities.
References 1. Palmer WE, Bloch SM, Chew FS. Menetrier disease. Am J Roentgenol 1992;158:62. 2. Sundt TM III, Compton CC, Malt RA. Menetrier’s disease. A trivalent gastropathy. Ann Surg 1988;208:694 –701. 3. Wolfsen HC, Carpenter HA, Talley NJ. Menetrier’s disease: a form of hypertrophic gastropathy or gastritis? Gastroenterology 1993;104:1310 –1319. For submission instructions, please see the Gastroenterology website (http://www.gastrojournal.org).
contacting the ABP. Additional information including eligibility requirements may also be found on the ABP website. Each applicant will be considered individually and must be acceptable to the ABP.
NOTICES
1419
Contact: American Board of Pediatrics, 111 Silver Cedar Court, Chapel Hill, North Carolina 27514-1513; call (919) 929-0461; fax (919) 918-7114 or (919) 929-9255; or visit the website at www. abp.org.
Answer to the Image of the Month Question (page 968): Menetrier Disease With Premalignant Transformation Upper gastrointestinal endoscopy showed large thickened rugal folds projecting into the gastric lumen (Figure A). There were multiple associated large polypoid lesions distributed throughout the body, fundus, and cardia of stomach. There was characteristic, relative sparing of the gastric antrum. Abdominal computerized tomography (CT) demonstrated diffuse thickening of the gastric wall in the form of rugal fold enlargement (Figure B). A maximum wall thickness of 4 cm was measured. The disease distribution noted on endoscopy correlated well with CT findings. The gastric lumen appeared diminutive and filled with giant rugal folds. Small bowel wall-thickening was seen in keeping with the typical protein-losing enteropathy and the patient’s known hypoalbuminemic status. Gastric biopsy demonstrated extensive foveolar hyperplasia with multifocal high-grade dysplasia. No definite carcinoma was seen. The patient had a total gastrectomy to eliminate the real risk of progression to gastric carcinoma and to prevent further protein loss. The pathological diagnosis was Menetrier disease with multifocal high-grade dysplasia. Menetrier disease (hypertrophic gastropathy) is a rare, idiopathic condition, seen most commonly in the fourth to sixth decade of life, and is characterized by hyperplasia of gastric mucosal epithelium. There is characteristic mucus hypersecretion leading to a negative nitrogen balance and hypoproteinemic state, and frequently, there is hyposecretion of acid. The incidence of progression to gastric carcinoma is estimated at 2%–15%. In patients not responding to medical therapy, total gastrectomy should be considered. The differential diagnosis for the endoscopic and CT appearances includes lymphoma, Zollinger–Ellison syndrome, amyloidosis, eosinophilic gastritis, and infiltrative neoplastic entities.
References 1. Palmer WE, Bloch SM, Chew FS. Menetrier disease. Am J Roentgenol 1992;158:62. 2. Sundt TM III, Compton CC, Malt RA. Menetrier’s disease. A trivalent gastropathy. Ann Surg 1988;208:694 –701. 3. Wolfsen HC, Carpenter HA, Talley NJ. Menetrier’s disease: a form of hypertrophic gastropathy or gastritis? Gastroenterology 1993;104:1310 –1319. For submission instructions, please see the Gastroenterology website (http://www.gastrojournal.org).