Juvenile polyposis of the stomach—a novel cause of hypergastrinemia

Abstract

Continuous acid-suppressive therapy, prokinetic therapy and total parenteral nutrition. Repetitive endoscopic polypectomy (also known as debulking) was performed twice and was followed by gastrectomy with duodenoesophageal anastomosis.

Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

“…Elevations can be appropriate (i.e., physiological), in that it is the normal physiological response to hypo-/achlorhydria; inappropriate in which hypergastrinemia is associated with hyperchlorhydria, as occurs in ZES, or due to a gastrin assay error [13, 17, 45, 54–56] (Table 2). Appropriate hypergastrinemic conditions such as chronic gastritis, pernicious anemia, pan-gastritis-associated H. pylori infections and ingestion of potent gastric acid antisecretory drugs (especially PPI’s) are by far the most common causes of fasting hypergastrinemia and thus they need to be differentiated from other inappropriate causes such as ZES.…”

Giant Stomach Secondary to Juvenile Polyposis Syndrome

“…1 Since the first case of gastric juvenile polyposis was reported in 1979, 5 only 25 cases have been documented. [2][3][4][5][6][7][8][9][10][11][12][13][14][15] This case is the fourth reported case of JPS causing gastric outlet obstruction. Unlike three of the previous case reports, gastric outlet obstruction resulted in massive dilatation of the stomach and was accompanied by hematemesis requiring multiple blood transfusions and hypoproteinemia.…”

Massive gastric juvenile‐type polyposis: a clinicopathological analysis of 22 cases

“…SMAD4 mutation appears to be the greatest risk factor for upper‐tract involvement, as up to 86% of affected patients with a germline SMAD4 mutation will have extracolonic involvement . A mutation in this gene also appears specifically to confer a risk of developing gastric polyps or gastric polyposis, unlike BMPR1A mutations; several reported cases of massive gastric polyposis have confirmed a SMAD4 mutation in the affected patient, although at least one patient was repeatedly negative for SMAD4 and BMPR1A mutations . Similarly, a SMAD4 mutation was confirmed in five of our cases, whereas molecular testing was negative in a sixth via Sanger sequencing analysis and multiplex ligation‐dependent probe amplification analysis for gross deletions or duplications.…”

“…Although that organ does not often bear the brunt of JPS, rare patients develop massive gastric polyposis, with a large portion of the gastric volume being occupied by several polyps; this type of polyposis can also occur in patients not formally diagnosed with JPS. This has been reported sporadically in the clinical and surgical literature, mostly in the form of case reports . It has been little explored in the English‐language pathology literature, aside from a case series/literature review totalling 12 patients from 1997 and mention of four patients with a ‘huge [gastric] polyp burden’ in a recent review of JPS of the upper gastrointestinal tract .…”