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Immunoglobulin g4-related hypertrophic pachymeningitis with skull involvement.

Lee YS, Lee HW, Park KS, Park SH, Hwang JH - Brain Tumor Res Treat (2014)

Bottom Line: Immunohistochemical staining showed markedly increased IgG4 (+) plasma cells.Since then, steroids and immunosuppressant medications were started.Follow-up MR imaging at 3 months after medication initiation demonstrated complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Kyungpook National University School of Medicine, Daegu, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

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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Figure 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).

Mentions: Histopathological findings of the mass demonstrated diffuse inflammatory cell infiltration, especially numerous plasma cells and lymphocytes in the sclerotic background (Fig. 2A, B). And involved skull also revealed lymphoplasmacytic infiltration and fibrosis (Fig. 2D). We consequently diagnosed idiopathic hypertrophic intracranial pachymeningitis and started steroid treatment. Four months after surgery, follow-up MR imaging revealed thinning and shrinking of the enhanced dura.


Immunoglobulin g4-related hypertrophic pachymeningitis with skull involvement.

Lee YS, Lee HW, Park KS, Park SH, Hwang JH - Brain Tumor Res Treat (2014)

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4231624&req=5

Figure 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).
Mentions: Histopathological findings of the mass demonstrated diffuse inflammatory cell infiltration, especially numerous plasma cells and lymphocytes in the sclerotic background (Fig. 2A, B). And involved skull also revealed lymphoplasmacytic infiltration and fibrosis (Fig. 2D). We consequently diagnosed idiopathic hypertrophic intracranial pachymeningitis and started steroid treatment. Four months after surgery, follow-up MR imaging revealed thinning and shrinking of the enhanced dura.

Bottom Line: Immunohistochemical staining showed markedly increased IgG4 (+) plasma cells.Since then, steroids and immunosuppressant medications were started.Follow-up MR imaging at 3 months after medication initiation demonstrated complete remission.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Kyungpook National University School of Medicine, Daegu, Korea.

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.

No MeSH data available.


Related in: MedlinePlus