Giant igg4-related pseudotumor of the esophagus resected with endoscopic submucosal dissection: a case report and review of the literature

Abstract

IgG4-related disease (IgG4-RD) is a systemic autoimmune disorder that has been defined in various organs. The disease is characterized by typical clinicopathological features including a dense lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis, and often an elevated serum IgG4 level. Esophageal IgG4-RD is rare, and its presentation as a solid mass is even more rare. Only 15 previous cases of IgG4-related esophageal disease have been described. We herein present a case of giant IgG4-related pseudotumor of the esophagus resected with endoscopic submucosal dissection (ESD) and a review of the literature. The patient was a 67-year-old man who was admitted to our hospital for assessment of progressive dysphagia. Upper gastrointestinal endoscopy revealed a 9 cm mass in the cervical esophagus. In the previous two hospitals, the patient’s mass could not be diagnosed despite repeated biopsies. Because of concerns regarding malignancy, endoscopic submucosal dissection was performed. Histopathological examination showed dense lymphoplasmacytic infiltration with predominant IgG4-positive plasma cells on a sclerotic background. The patient was diagnosed with IgG4-RD. During the follow-up, no residual mass was detected but the patient was diagnosed with lung adenocarcinoma. We present a unique case of giant IgG4-related pseudotumor of the esophagus. Resection with ESD of such a big mass of IgG4-RD in the esophageal region has never been reported before in the literature.

IgG4-Related Oesophageal Disease with Cytomegalovirus Infection: A Case Report

“…The oesophageal involvement of IgG4-RD (IgG4-RDE) has been uncommonly reported in the literature. Nese et al [ 1 ] reported that only 15 cases in the literature have previously described the oesophageal involvement of IgG4-RDE. CMV-associated gastrointestinal diseases usually occur in immunocompromised patients; however, a few cases have also been described in healthy hosts [ 19 ].…”

“…Previous reports indicated that IgG4-RDE is more frequent in males and occurs most often in elderly patients. The clinical symptoms of IgG4-RDE primarily include dysphagia and weight loss, and, more rarely, odynophagia, epigastric pain, and acid reflux [ 1 ]. Gastric endoscopies can reveal ulcerations and strictures, or mass-like lesions, mainly involving the distal and mid-oesophagus [ 21 ].…”

“…IgG4-related disease is a specific type of chronic autoimmune disease. The symptoms of IgG4-related disease are diverse and complex, and they are often accompanied by elevated serum IgG4 concentrations, with typical clinicopathological features, including dense infiltration of lymphocytes/plasma cells and fibrosis, large numbers of IgG4-positive plasma cells in the affected organs, storiform fibrosis, and obliterative phlebitis, which are reported to be the unique and characteristic features of IgG4-RD [ 1 , 2 , 3 ]. The disease can affect virtually any organ.…”