Korean J Radiol.  2014 Feb;15(1):66-71. 10.3348/kjr.2014.15.1.66.

Small Bowel Obstruction Caused by Peritoneal Immunoglobulin G4-Related Disease Mimicking Carcinomatosis: Case Report

• Coulier B
• Montfort L
• Beniuga G
• Pierard F
• Gielen I

• KMID: 1711479
• DOI: http://doi.org/10.3348/kjr.2014.15.1.66

Abstract

We hereby report a case of diffuse pelvic peritoneal involvement by immunoglobulin G4-related disease (IgG4-RD). Numerous pelvic masses and nodules showing delayed enhancement on enhanced abdominal CT were found to congregate in the pelvic organs of a 57-year-old female presenting with intestinal subocclusion. The differentiation between peritoneal IgG4-RD and pelvic peritoneal carcinomatosis was only made by histopathology and immunohistochemistry performed after surgical resection. Autoimmune pancreatitis represents the historical prototype of IgG4-RD, but the spectrum of manifestations involving various organs has expanded during the last decade. In this report, we shortly review this clinical entity.

Keyword

IgG4-related disease; IgG4; Abdomen, CT; Immunohistochemistry; Pathologic correlation

MeSH Terms

Carcinoma/diagnosis
Female
Humans
*Immunoglobulin G
Immunohistochemistry
Intestinal Obstruction/*etiology
*Intestine, Small
Middle Aged
Paraproteinemias/*complications/diagnosis/pathology
Peritoneal Diseases/*complications/diagnosis/pathology
Peritoneal Neoplasms/diagnosis
Immunoglobulin G

Figure

• 

  Fig. 1 57-year-old woman presenting with IgG4-related peritoneal disease. A-D.Four selected contrast-enhanced axial CT views of hypogastric and pelvic areas show diffuse infiltration of pelvic organs by enhancing masses which encase distal ileum (white arrows) causing intestinal subocclusion (grey stars). Diffuse small round nodules are also dispersed within peritoneal spaces (black arrows). White stars show uterus, black stars small amount of fluid in Douglas pouch and grey arrows ovaries. IgG4 = immunoglobulin G4 E. Gross anatomy of resected organs including uterus (white star), ovaries and portion of incarcerated ileum (black arrow). Organs are massively infiltrated by numerous irregular whitish nodules mimicking diffuse carcinomatosis (white arrows). F. Gross anatomy of 30 cm of resected ileum terminale. Numerous white nodules (white arrows) infiltrating mesenteric fat tissue. G. Section through ileum (black star) showing pseudotumoral fibrosing tissue (black arrows) infiltrating full thickness of intestinal wall. IgG4 = immunoglobulin G4 H. Photomicrograph (hematoxylin-eosin [H-E] stain; magnification, × 10) of section of fatty peritoneal tissue showing area of dense storiform fibrosis (white arrow) separated by dense inflammatory lymphoplasmacytic infiltrate (black arrows) with germinal center formation (small black arrows). Black star shows normal fat tissue. I. Photomicrographic (H-E stain; magnification, × 40) details of typical storiform fibrosis intermingled with lymphoplasmacytic inflammatory infiltrate. J. Photomicrographic (orcein stain; magnification, × 40) details of obliterative phlebitis. K. Photomicrograph (IgG4 immunostain; magnification, × 40) showing numerous IgG4-positive plasma cells. IgG4 = immunoglobulin G4

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