Erasing Giant Folds: Ménétrier's Disease Treated with Cetuximab

Excerpt

A 65-year-old man was evaluated for complaints of diarrhea, loss of appetite, and substantial (25 lbs) unintentional weight loss over several months. His past medical history was unremarkable. On initial evaluation he appeared cachectic, weighing 105 lbs, though the examination was otherwise unremarkable. Laboratory studies showed anemia (hemoglobin 9.7 g/dL), evidence of iron deficiency (ferritin 29.6 ng/mL, serum iron 12 µg/dL, transferrin saturation 5%), profound hypoalbuminemia (1.1 g/dL), low fecal elastase level (32 µg/g), and elevated qualitative fecal fat. Serum gastrin level was normal (41 pg/mL). Stool studies to exclude infectious causes of diarrhea (Giardia Ag, C. difficile toxin, ova & parasites, and stool culture) were unremarkable. CT scan of the abdomen (Fig. 1) and MR enterography (Fig. 2) showed thickening of the walls of the ileum, cecum, and ascending colon with additional duodenal and gastric antral wall thickening. A gold Quantiferon test for tuberculosis was negative. Esophagogastroduodenoscopy (EGD) revealed markedly thickened polypoid folds in the stomach with a large amount of overlying mucoid material (Fig. 3). Duodenal polyps and duodenal wall thickening were also identified. Colonoscopy revealed marked induration and pseudopolyp formation in the ascending colon of uncertain significance. Histology revealed foveolar hyperplasia in the stomach and inflammatory/hyperplastic changes in the colon. The patient was started on loperamide and pancreatic enzyme supplementation with only a modest improvement of diarrhea. Due to clinical concern for a hypertrophic gastric polypoid condition, repeat EGD using deeper snare biopsies revealed proliferation of foveolar epithelium with elongated, branched pits and dilated gastric glands. A reduction in chief and parietal cells was also noted (Fig. 4). Endoscopic ultrasound showed marked polypoid thickening of the gastric wall (Fig. 5). Immunostaining for cytomegalovirus (CMV) and H. pylori was negative (not shown). In the context of clinical and endoscopic findings, the histologic findings were compatible with Ménétrier's disease. The patient was started on long-acting octreotide (LAR 20 mg IM every 4 weeks), iron infusions, and total parenteral nutrition (TPN) that led to an initial improvement. Unfortunately, a few months later, his symptoms worsened, necessitating multiple hospitalizations for dehydration, multiple electrolyte abnormalities, and progressive weight loss. He was subsequently evaluated by medical oncology and started on IV cetuximab, with a loading dose of 400 mg/m² followed by 3 weekly infusions of 250 mg/m² followed by a maintenance dose of cetuximab (250 mg/m² every two weeks). The patient’s diarrhea and stool frequency decreased by > 50% and he was discharged. By the time of the last office visit 6 months later, the patient had regained his usual weight and was controlling his diarrhea with loperamide.

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