2018 Volume 51 Issue 10 Pages 599-606
We report a rare case of IgG4-related sclerosing disease presenting as a gastric submucosal tumor. An 85-year-old man was admitted to our hospital with a slightly enhanced tumor measuring 4 cm from the antrum of the stomach to the bulb of the duodenum detected by CT in a case of deterioration of diabetes mellitus. Upper gastrointestinal endoscopy showed a submucosal like tumor in the antrum of the stomach. Endoscopic US revealed a hypoechoic tumor in the fourth layer of the gastric wall. Endoscopic ultrasound-guided fine needle aspiration biopsy was unable to reveal definite diagnosis. Distal gastrectomy was performed under a preoperative diagnosis of gastrointestinal stromal tumor. Macroscopically, a light brownish solid and elastic hard mass was recognized on the cut surface of the specimen. Histologically, numerous lymphoid follicles were formed in the muscle layer, and infiltration of numerous plasma cells and fibrosis were recognized. Plasma cells in the tumor were positive for IgG and IgG4 (IgG4/IgG=100%). High levels of IgG4 were confirmed by postoperative blood tests. Based on these findings, we diagnosed IgG4-related sclerosing disease.