Unusual case of adult familial Menetrier disease in siblings

Abstract

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.

A case report of diffuse hyperplastic gastropathy with multiple polypoid formations in a patient with pernicious anemia, Helicobacter pylori infection, hypergastrinemia and hypoalbuminaemia: Do not forget of Ménétrier’s disease

“…Even though Menetrier’s disease usually affects mainly the fundus and body of the stomach, total gastrectomy has been preferred over partial gastrectomy to avoid the risk for symptoms persistence, and the risk for malignant transformation in the gastric remnant along lifetime [ 9 , 10 , [12] , [13] , [14] ]. For gastric cancer, the risk for postoperative severe complications seems to be higher in total gastrectomy [ 14 ] and quality of life [ 15 ] seems to be poorer in total gastrectomy.…”

Hypertrophic gastropathy associated with gastric sarcoma in a dog

“…Ménétrier disease is a hyperproliferative disorder of the stomach caused by a dysregulated receptor tyrosine kinase, which is attributed to overexpression of transforming growth factor–alpha (TGF-α) with excessive stimulation of gastric epidermal growth factor receptors in humans. 3 Although most cases are considered to be idiopathic, rare inherited or familial forms of this disease have been reported in people 2,6 and have been suspected in 3 Cairn Terrier littermates. 8…”

“…Ménétrier disease may cause abdominal pain, vomiting, and weight loss, and clinical signs may be present for years prior to diagnosis, 8,12 although it can also be asymptomatic. 10 Hypoproteinemia and anemia because of protein and blood leakage through the altered gastric mucosa have been reported in people with Ménétrier disease 2,3,5,12 and dogs with Ménétrier-like disease, alone or in association with gastric carcinoma. 7,11,13 Although our case was not hypoproteinemic, the dog had mild anemia associated with iron deficiency.…”