Primary Gastric Linitis Plastica Presenting as Severe Non-Healing Helicobacter pylori-Associated Gastritis

Abstract

Purpose: Gastric linitis plastica (GLP) is a rigid and constrictive thickening of the stomach secondary to diffuse infiltration of poorly differentiated adenocarcinoma. The risk factors leading to GLP are poorly understood, and believed to be secondary to underlying genetic predispositions, i.e. E-cadherin mutations. In contrast to intestinal-type gastric adenocarcinomas, diffuse-type GLP is not believed to be related to environmental risk factors, and has not been described as associated with Helicobacter pylori. We describe a case of GLP associated with, and presenting as severe H.pylori-associated gastritis. A 48 year old man with no past medical history was admitted for two weeks of new abdominal fullness with nausea/vomiting. CT abdomen/pelvis demonstrated dilated stomach with H. pyloric thickening. On upper endoscopy, several antral ulcers with surrounding severe pyloric congestion and edema were noted. Antral biopsies showed moderate chronic gastritis with marked reactive changes, and serum H. pylori IgG antibodies were positive. The patient was diagnosed with, and treated for severe H. pylori gastritis with ulcerative inflammation causing partial gastric outlet obstruction. The patient demonstrated slow clinical improvement, and was taken back to endoscopy at two weeks. Persistent ulcerations and congestion was noted, and endoscopic ultrasound (EUS) exam demonstrated diffuse infiltration of the gastric wall with loss of normal five layer anatomy. Pathology from deep bite biopsies and fine needle aspiration showed diffuse poorly differentiated adenocarcinoma. The patient was taken for a subtotal gastrectomy and discharged for outpatient adjuvant chemoradiation. The diffusely infiltrative nature of GLP leads to a rigid stomach that typically manifests endoscopically with a “leather bottle” appearance, and clinically as epigastric pain with nausea/vomiting, anorexia, and weight loss. This type of gastric cancer has a very poor prognosis, occurs in relatively young patients, and has not been associated with H. pylori gastritis and other environmental risk factors. In this unique case, the patient presented with non-healing ulcers with associated partial outlet obstruction that was attributed to an underlying H. pylori infection. This case highlights a number of salient issues: 1.) the inherent difficult diagnosis of GLP due to high frequency of mucosal sparing and false negative biopsies, 2.) the need to re-evaluate non-healing ulcers or patients with H. pylori not responding to traditional therapy, and 3.) the need for re-evaluation of a possible link between H. pylori and GLP.