J Korean Soc Radiol.  2015 Aug;73(2):108-112. 10.3348/jksr.2015.73.2.108.

Tumefactive Immunoglobulin G4-Related Disease Involving the Dura Mater: A Case Report and Literature Review

• Lee JH
• Lee JY
• Park DW
• Ko Y
• Paik SS
• Lee YJ

• KMID: 1964261
• DOI: http://doi.org/10.3348/jksr.2015.73.2.108

Abstract

Immunoglobulin G4 (IgG4)-related disease is a well-known disorder characterized by an inflammatory reaction with an increase in the number of IgG4-positive plasma cells associated with sclerosis. IgG4-related disease often affects the dura mater with a pattern of diffuse thickening when the central nervous system is involved. However, some nodular dural thickening requires discrimination from tumors because of obviously different treatment options. We report of a case of IgG4-related disease with tumefactive dural involvement.

MeSH Terms

Central Nervous System
Discrimination (Psychology)
Dura Mater*
Immunoglobulins*
Meningioma
Meningitis
Plasma Cells
Sclerosis
Immunoglobulins

Figure

• 

  Fig. 1 Computed tomography (CT) and magnetic resonance imaging of immunoglobulin G4-related disease with tumefactive dural involvement in a 57-year-old man. Initial non-enhanced CT (A) demonstrates a slightly hyperdense mass (black arrow) along the anterior falx. The mass (black arrow) shows intermediate signal intensity on T1- (B) and low signal intensity on T2-weighted images (C) with extensive perilesional parenchymal edema. A post-contrast T1-weighted image (D) shows, diffuse thickening and enhancement of dura mater (white arrow) at the anterior falx cerebri and parasagittal region. The mass shows homogeneous enhancement with a jagged border and co-involvement of adjacent brain parenchyma (white arrowhead).

• 

  Fig. 2 Immunoglobulin G4 (IgG4) immunohistochemical staining (× 400) shows infiltration of abundant IgG4-positive plasma cells (white arrows).

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