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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

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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Figure 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.

Mentions: Eight months after surgery, a partial seizure of the right upper arm developed. Brain MRI revealed a new mass adjacent to the previous one, and diffuse dura enhancement. Recurrence was suspected, and re-excision was considered (Fig. 3A, B). However, we decided to perform additional immunohistochemical staining of immunoglobulin G (IgG) and IgG4 for the previous specimen, because it was suggested that an IgG4-related sclerosing disease might represent a component of idiopathic hypertrophic pachymeningitis. Immunohistochemical studies revealed increased both IgG4 (+) plasma cells (66/HPF) and IgG4+/IgG+ cell ratio (57% in the hot spot) (Fig. 3C). Also obliterative phlebitis was further recognized (Fig. 2C). The histological features of dense lymphoplasmacytic infiltration, fibrosis and obliterative phlebitis in conjunction with the immunohistochemical results support the diagnosis of highly suggestive of IgG4-related hypertrophic pachymeningitis. But patient's serum IgG4 concentration is not elevated. Then the patient took continuous oral prednisolone and immunosuppressants such as either azathioprine or cyclophosphamide. Brain MRI obtained at 3 months after immunosuppressant medication demonstrated that the enhanced mass and thickened dura had disappeared (Fig. 3D). The patient has since been doing very well, without any significant symptoms suggestive of disease recurrence.


Immunoglobulin g4-related hypertrophic pachymeningitis with skull involvement.

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

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

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

Figure 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.
Mentions: Eight months after surgery, a partial seizure of the right upper arm developed. Brain MRI revealed a new mass adjacent to the previous one, and diffuse dura enhancement. Recurrence was suspected, and re-excision was considered (Fig. 3A, B). However, we decided to perform additional immunohistochemical staining of immunoglobulin G (IgG) and IgG4 for the previous specimen, because it was suggested that an IgG4-related sclerosing disease might represent a component of idiopathic hypertrophic pachymeningitis. Immunohistochemical studies revealed increased both IgG4 (+) plasma cells (66/HPF) and IgG4+/IgG+ cell ratio (57% in the hot spot) (Fig. 3C). Also obliterative phlebitis was further recognized (Fig. 2C). The histological features of dense lymphoplasmacytic infiltration, fibrosis and obliterative phlebitis in conjunction with the immunohistochemical results support the diagnosis of highly suggestive of IgG4-related hypertrophic pachymeningitis. But patient's serum IgG4 concentration is not elevated. Then the patient took continuous oral prednisolone and immunosuppressants such as either azathioprine or cyclophosphamide. Brain MRI obtained at 3 months after immunosuppressant medication demonstrated that the enhanced mass and thickened dura had disappeared (Fig. 3D). The patient has since been doing very well, without any significant symptoms suggestive of disease recurrence.

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