Clivus-involved immunoglobulin G4 related hypertrophic pachymeningitis mimicking meningioma: A case report

Abstract

Background: Immunoglobulin G4 related disease (IgG4-RD) is a fibroinflammatory disease with markedly elevated serum IgG4 levels and fibrous tissue proliferation, accompanied by numerous plasma cells. IgG4 related hypertrophic pachymeningitis (IgG4-RHP) is relatively rare and indistinguishable from other phymatoid diseases before the operation. The risk of long-term immunosuppression needs to be balanced with disease activity.

Case summary: A 40-year-old man presented with headache and bilateral abducent paralysis. He was also diagnosed with pulmonary tuberculosis 10 years ago and was on regular treatment for the same. Before the operation and steroid therapy, the patient was suspected of having tubercular meningitis at a local hospital. A clivus lesion was found via brain magnetic resonance imaging (MRI) at this presentation. He was preliminarily diagnosed with meningioma and underwent Gamma Knife Surgery. Transnasal endoscopic resection was performed to treat deterioration of nerve function. Postoperative pathologic examination suggested IgG4-RD. Moreover, the serum IgG4 was elevated at 1.90 g/L (reference range: 0.035-1.500 g/L). After steroid therapy for 2 mo, the lesion size diminished on MRI, and the function of bilateral abducent nerves recovered.

Conclusion: IgG4-RHP is relatively rare and indistinguishable before the operation. Elevated serum IgG4 levels and imaging examination help in the diagnosis of IgG4-RHP. Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit.

Keywords: Case report; Clivus; Hypertrophic pachymeningitis; Immunoglobulin G4 related disease; Immunoglobulin G4 related hypertrophic pachymeningitis.

Figure 1

Lesion alteration on the sagittal view. A: This image shows the condition when the patient was first admitted to the tuberculosis department diagnosed as having a meningioma (December 2016); B: Before the performance of Gamma Knife Surgery (March 2017); C: Six months after the performance of Gamma Knife Surgery (September 2017); D: Preoperative image (November 2019); E: Postoperative image (January 2020); F: Two months after operation (March 2020).

Figure 2

Examination of visual field before and after the operation. The alteration of the visual field before and after the operation is shown. The second row is the result at 4 mo after the operation.

Figure 3

Pathological features of the resection part indicating the diagnosis of immunoglobulin G4 related hypertrophic pachymeningitis. A and B: Proliferation of fibrous tissue accompanied by numerous lymphocytes and plasma cells as shown by hematoxylin and eosin staining (A: × 100; B: × 400); C: Immunohistochemical staining exhibited an increased number of CD138-positive plasma cells (× 400); D: Large number of immunoglobulin G4-positive plasma cells as shown by immunohistochemical staining [~200 per high power field (× 400)].