IgG4-related disease presenting as a submucosal tumor of the stomach resected with laparoscopic endoscopic cooperative surgery: a case report

Abstract

Background IgG4-related disease (IgG4-RD) is an immune-mediated disorder in which abundant IgG4-positive plasma cells infiltrate affected organs. There have been reported four cases of probable IgG4-RD presenting as a submucosal tumor of the stomach. We herein report the first case of definite IgG4-RD presenting as a submucosal tumor of the stomach resected with laparoscopic endoscopic cooperative surgery (LECS). Case presentation A 70-year-old woman with a 6-year history of autoimmune pancreatitis was referred to our department because a 15-mm submucosal tumor in the greater curvature of the lower part of the stomach had been identified via upper gastrointestinal endoscopy. Endoscopic ultrasonography showed a 10-mm low-echoic lesion derived from the submucosal layer of the stomach. A fine-needle aspiration biopsy was attempted, but the tumor was too hard for sampling. F-fluorodeoxyglucose (FDG) positron emission tomography showed an FDG uptake, suggesting a possibility of malignant disease. As the diagnosis could not be confirmed, LECS for both the diagnosis and curative treatment was performed. A histopathological examination showed a tumor with IgG4-positive lymphoplasmacytic infiltration and fibrosis. The ratio of IgG4+/IgG+ lymphoplasmacytic cells was > 80%. A laboratory examination showed elevation of the serum IgG4 levels preoperatively. Thus, the final diagnosis was IgG4-RD of the stomach. No recurrence was observed within 1 year after surgery. Conclusions We encountered a case of definite IgG4-RD presenting as a gastric SMT in which a correct diagnosis was achieved by a minimally invasive LECS technique. IgG4-RD may present as a gastric lesion and should be taken into consideration as a differential diagnosis.

Utility of gastric biopsy in diagnosing IgG4‐related gastrointestinal disease

“…IgG4‐related gastrointestinal (GI) disease (IgG4‐GID) is rare, but it has been reported anecdotally. In fact, various clinical manifestations, such as ulcerations, 1–5 polyps, 6–8 wall thickening, 9–16 submucosal tumors (SMTs), 17–27 GI obstruction, 28–30 vasculitis, 31 fistulas, 32,33 and the infiltration of IgG4‐positive plasma cells without morphologic abnormalities, 34 have been reported in IgG4‐GID. As a matter of fact, most cases of IgG4‐GID do not exhibit other organ involvement as observed in IgG4‐RD cases; thus, it is unlikely that all these cases represent genuine IgG4‐GID.…”

Clinicopathological characteristics of gastric IgG4‐related disease: Systematic scoping review

“…Table 2 presents the baseline demographics, diagnostic findings, and chief clinical features of gastric IgG4‐RD from the individual cases ( n = 42) 7–12,17–47 . There were slightly more male cases than female cases (59.5% male).…”

IgG4-Related Disease With Gastrointestinal Involvement: Case Reports and Literature Review

“…Table 1 shows the cases of IgG4-related gastric disease reported in articles available from PUBMED ( 18 – 28 ). Most patients had tumors and were diagnosed by pathology after surgery, and the disease may be related to suppressor T cells.…”