Obatake Masayoshi, Sato Koichi, Yagi Shigehiko, Ohtani Hiromi, Kito Katsumi
Department of Surgery, Ehime Prefectural Central Hospital, 83 Kasugamachi, Matsuyama, Ehime, 790-0024, Japan.
Department of Pathology, Ehime Prefectural Central Hospital, Ehime, Japan.
Surg Case Rep. 2021 Feb 5;7(1):40. doi: 10.1186/s40792-021-01126-6.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder that can involve multiple organs. It is characterized by IgG4-positive plasma cell-rich storiform fibrosis and obliterative phlebitis associated with a high serum IgG4 level. There are few reports of gastric IgG4-RD, especially those detected prior to systemic or other organ involvement.
A 70-year-old man was diagnosed with type 0-IIc gastric cancer at the anterior wall of the gastric corpus by upper gastrointestinal endoscopy. In addition, a submucosal tumor (SMT) 7 mm in diameter was found at the greater curvature of the angulus. Laparoscopic distal gastrectomy with regional lymph node dissection was performed. Pathology revealed a poorly differentiated adenocarcinoma in the type 0-IIc lesion and storiform fibrosis with infiltration of a large number of IgG4-positive plasma cells in the SMT. Postoperative laboratory testing showed elevation of serum IgG4 levels; thus, we diagnosed the SMT as IgG4-RD. Intriguingly, the gastric IgG4-RD lesion demonstrated IgG4-positive plasma cell-rich arteritis as well as typical obstructive phlebitis. The patient has been followed for 2 years after surgery without recurrence of cancer, but skin lesions of IgG4-RD have appeared.
We report a rare case of IgG4-RD presenting as a gastric SMT, accompanied by early-stage gastric cancer. Our case may support a newly proposed relationship between IgG4-RD and malignancies. The gastric IgG4-RD lesion showed arteritis as well as obliterative phlebitis, potentially providing novel insight into IgG4-related vascular lesions.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种可累及多个器官的免疫介导性炎症性疾病。其特征为富含IgG4阳性浆细胞的席纹状纤维化和闭塞性静脉炎，同时伴有血清IgG4水平升高。胃IgG4-RD的报道较少，尤其是在系统性或其他器官受累之前被检测到的病例。
一名70岁男性经上消化道内镜检查诊断为胃体前壁0-IIc型胃癌。此外，在胃角大弯处发现一个直径7毫米的黏膜下肿瘤（SMT）。实施了腹腔镜远端胃切除术及区域淋巴结清扫术。病理显示0-IIc病变为低分化腺癌，SMT中存在席纹状纤维化并伴有大量IgG4阳性浆细胞浸润。术后实验室检查显示血清IgG4水平升高；因此，我们将SMT诊断为IgG4-RD。有趣的是，胃IgG4-RD病变显示出富含IgG4阳性浆细胞的动脉炎以及典型的闭塞性静脉炎。该患者术后已随访2年，无癌症复发，但出现了IgG4-RD的皮肤病变。
我们报告了一例罕见的以胃SMT形式出现的IgG4-RD病例，同时伴有早期胃癌。我们的病例可能支持IgG4-RD与恶性肿瘤之间新提出的关系。胃IgG4-RD病变显示出动脉炎以及闭塞性静脉炎，可能为IgG4相关血管病变提供新的见解。
