Korean J Gastroenterol.  2012 Oct;60(4):258-261. 10.4166/kjg.2012.60.4.258.

A Case of Intraperitoneal Immunoglobulin G4-related Inflammatory Pseudotumor

Affiliations
  • 1Department of Internal Medicine, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea. jbkim87@hallym.or.kr
  • 2Department of Pathology, Kangnam Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea.

Abstract

The term inflammatory pseudotumor (IPT) has been used to describe inflammatory and fibrosing tumoral processes of an undetermined cause that may involve a variety of organ system. IgG4-related disease is a newly recognized fibroinflammatory condition characterized by IgG4-producing plasma cell expansion in affected organs and, often but not always, elevated serum IgG4 concentrations. IgG4-related IPTs, a subtype of IPT, are characterized by dense infiltration of IgG4-positive plasma cells and stromal fibrosis. The association between inflammatory pseudotumor and IgG4 was first reported with a regard to sclerosing pancreatitis. Despite there are many reports on intraperitoneal IPTs including both cellular and lymphoplasmacytic type, only a few cases have been confirmed to be IgG4-related. We experienced a case of intraperitoneal IgG4-related inflammatory pseudotumor in an 83-year-old woman presenting with epigastric pain and malaise. Surgical specimens revealed an IgG4-related inflammatory pseudotumor.

Keyword

IgG4-related disease; Immunoglobulin G4; Inflammatory pseudotumor; Intraperitoneal tumor

MeSH Terms

Aged, 80 and over
C-Reactive Protein/analysis
Female
Granuloma, Plasma Cell/*diagnosis/pathology/surgery
Humans
Immunoglobulin G/*blood
Plasma Cells/metabolism
Positron-Emission Tomography
Tomography, X-Ray Computed

Figure

  • Fig. 1 CT with contrast enhancement. An 11×8 cm sized well defined heterogenous mass adjacent to the stomach was noted.

  • Fig. 2 Gross specimen. The well circumscribed mass measured 10.5×6.5×3.5 cm.

  • Fig. 3 Histologic features of the resected specimen. (A) The lymphoplasmacytic infiltrate showed the formation of germinal centers (H&E, ×200). High-power view showed an admixture of spindle-shaped cells and prominent lymphoplasma cells within fibrotic stroma (inset, H&E, ×400). (B) Immunohistochemical staining of IgG4. Up to 25/HPF IgG4 positive plasma cells were noted (×400).


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