Brain Tumor Res Treat.  2014 Oct;2(2):87-91. 10.14791/btrt.2014.2.2.87.

Immunoglobulin G4-Related Hypertrophic Pachymeningitis with Skull Involvement

• Lee YS
• Lee HW
• Park KS
• Park SH
• Hwang JH

• KMID: 2134277
• DOI: http://doi.org/10.14791/btrt.2014.2.2.87

Abstract

Immunoglobulin G4 (IgG4)-related hypertrophic pachymeningitis, defined as focally or diffusely thickened dura mater and lymphoplasmacytic infiltration with increased IgG4 bearing plasma cells, is a rare disease. Moreover, cases involving bone are even rarer. In this report, the authors describe a case of IgG4-related hypertrophic pachymeningitis involving the skull in a 65-year-old man presenting with generalized tonic seizures. There is a 2.4 cm diameter extra-axial mass at the vertex of the left frontal convexity and thickened dura mater with contrast enhancement on magnetic resonance (MR) imaging. In addition, the skull adjacent to the mass was focally enhanced. He underwent surgical resection of the enhanced mass and skull. Histopathological findings revealed chronic inflammation with fibrosis, and idiopathic hypertrophic intracranial pachymeningitis was considered. However, eight months after surgery, partial seizures developed and brain MR imaging revealed a recurrence adjacent to the previous mass. We decided to perform additional immunohistochemical staining of the previous specimen, instead of a re-excision. Immunohistochemical staining showed markedly increased IgG4 (+) plasma cells. Consequently, IgG4-related hypertrophic meningitis was confirmed. Since then, steroids and immunosuppressant medications were started. Follow-up MR imaging at 3 months after medication initiation demonstrated complete remission. In conclusion, IgG4-related hypertrophic pachymeningitis should be considered in the differential diagnosis of hypertrophic cranial pachymeningitis.

Keyword

Immunoglobulin G4-related disease; Skull; Magnetic resonance imaging; Immunosuppressants

MeSH Terms

Aged
Brain
Diagnosis, Differential
Dura Mater
Fibrosis
Follow-Up Studies
Humans
Immunoglobulin G
Immunoglobulins*
Immunosuppressive Agents
Inflammation
Magnetic Resonance Imaging
Meningitis*
Plasma Cells
Rare Diseases
Recurrence
Seizures
Skull*
Steroids
Immunoglobulin G
Immunoglobulins
Immunosuppressive Agents
Steroids

Figure

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  Fig. 1 Preoperative magnetic resonance (MR) imaging. A and B: T1- and T2-weighted MR images revealing a hypodense mass at the left frontal convexity (arrowheads). C and D: Contrast enhanced MR images showing a homogenously enhanced mass at the vertex of the left frontal convexity combined with a diffusely thickened and enhanced dura mater and enhanced skull along the left hemisphere.

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  Fig. 2 Histopathological findings (hematoxylin and eosin-stain). A: Microscopic findings of the mass and skull demonstrating florid inflammatory cell infiltrates and storiform fibrosis (×40). B: The inflammatory cells, which consist of plasma cells, lymphocyte and some neutrophils (×400). C: Nearly obliterated vein by inflammatory cell aggregation (×200). D: The skull revealing lymphoplasmacytic infiltration and fibrosis (×40).

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  Fig. 3 Postoperative magnetic resonance (MR) imaging and immunohistochemical staining. A and B: Eight months after surgery, contrast enhanced MR images showing the newly enhanced lesion adjacent to the previous mass. C: Immunohistochemical staining of immunoglobulin G (IgG) (left) and immunoglobulin G4 (IgG4) (right) (×400). D: Three months after immunosuppressant medication, brain MR imaging demonstrating that the enhanced mass and thickened dura had disappeared.

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