J Pathol Transl Med.  2016 Jan;50(1):67-70. 10.4132/jptm.2015.07.28.

IgG4-Related Disease Presented as a Mural Mass in the Stomach

• Woo CG
• Yook JH
• Kim AY
• Kim J

• KMID: 2211405
• DOI: http://doi.org/10.4132/jptm.2015.07.28

Abstract

Isolated gastric IgG4-related disease (IgG4-RD) is a very rare tumefactive inflammatory condition, with only a few cases reported to date. A 48-year-old woman was incidentally found to have a subepithelial tumor in the stomach. Given a presumptive diagnosis of gastrointestinal stromal tumor or neuroendocrine tumor, she underwent wedge resection. The lesion was vaguely nodular and mainly involved the submucosa and proper muscle layer. Microscopically, all classical features of type I autoimmune pancreatitis including lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and numerous IgG4-positive plasma cells were seen. She had no evidence of IgG4-RD in other organs. Although very rare, IgG4-RD should be considered one of the differential diagnoses in the setting of gastric wall thickening or subepithelial mass-like lesion. Deep biopsy with awareness of this entity might avoid unnecessary surgical intervention.

Keyword

Immunoglobulin G4; Stomach; Autoimmune diseases; Granuloma, plasma cell

MeSH Terms

Autoimmune Diseases
Biopsy
Diagnosis
Diagnosis, Differential
Female
Fibrosis
Gastrointestinal Stromal Tumors
Granuloma, Plasma Cell
Humans
Middle Aged
Neuroendocrine Tumors
Pancreatitis
Phlebitis
Plasma Cells
Stomach*

Figure

• 

  Fig. 1. Endoscopic and abdominal computed tomography scan images. (A) Localized smooth elevation of the gastric mucosa without mucosal fold abnormality. (B) A well-defined, solid, enhancing mass measuring 3.6 × 2.2 cm at the posterior wall of the stomach midbody (arrow).

• 

  Fig. 2. Gross and microscopic appearance of the resected specimen. (A) An ill-defined, yellowish grey mass involves the full thickness of the gastric wall except the mucosa. (B) The mass is not encapsulated and is filled with fibrotic tissue and multiple lymphoid follicles. (C) Storiform fibrosis is observed between lymphoid follicles. (D) Numerous plasma cells and many eosinophils are noted in the fibrotic stroma. (E) Obliterative phlebitis is demonstrated in elastic staining (arrow). Note the residual elastic fiber of the obliterated vein (Van Gieson). (F) Numerous IgG4-positive cells are noted in the sclerotic area.

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