Post written by Suchapa Arayakarnkul, MD, from the Department of Medicine, University of Minnesota, Minneapolis, and Daniela Guerrero Vinsard, MD, from the Division of Gastroenterology and Hepatology, Minneapolis VA Medical Center, Minneapolis, and Division of Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, Minnesota, USA.

We present a case of gastric adenocarcinoma and proximal polyposis syndrome (GAPPS) and review its diagnostic criteria and management.

We aimed to highlight the pathognomonic signs of this rare genetic cancer syndrome, as early recognition and timely preventive management are crucial for improving patients’ long-term survival.

Fundic gland polyps may represent a benign finding in some patients; however, when present in large numbers—particularly >100—they should raise endoscopists’ suspicion for GAPPS. Additional concerning features include polyp sparing of the antrum, duodenum, and colorectum; a family history of dysplastic fundic gland polyps or gastric adenocarcinoma; and presence of a mutation in promoter 1B of the adenomatous polyposis coli gene.

Importantly, GAPPS is a distinct entity from familial adenomatous polyposis, as underlying adenomatous polyposis coli gene mutations involve different regions. To date, treatment strategies are individualized, ranging from endoscopic surveillance to risk-reducing surgical resection. However, no standardized recommendations exist regarding postsurgical surveillance, and further studies are needed to guide management.

A, Esophagogastroduodenoscopy showing innumerable medium-sized sessile polyps in the gastric fundus. B, Esophagogastroduodenoscopy with the retroflexion view visualizing the fundus with polyps. There was complete antral and small bowel sparing. C, Laparoscopic total gastrectomy was performed. Dissection of the resected stomach confirmed the presence of innumerable polyps.

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