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Lymphoplasmacyte-Rich Meningioma or IgG4-Related Disease: Walking the Thin Line
Ariba Zaidi1, Debajyoti Chatterjee1, Bishan Radotra1
1Department of Histopathology, Postgraduate Institute of Medical Education and Research, Sector-12, Chandigarh, India.
Insights
Lymphoplasmacyte-rich meningioma, a rare tumor, presents with dense inflammation that can mimic other conditions. This case suggests a potential link between this meningioma subtype and IgG4-related disease, warranting further investigation.
Area of Science:
- Neuropathology
- Immunopathology
Background:
- Lymphoplasmacyte-rich meningioma (LP meningioma) is a rare WHO grade 1 meningioma variant.
- Its dense lymphoplasmacytic infiltrate can obscure the neoplastic meningothelial cells, mimicking inflammatory lesions.
Observation:
- A case of LP meningioma in a 47-year-old female is presented, located in the left parasagittal region.
- Histological examination revealed significant lymphoplasmacytic infiltrate, dense fibrosis, and numerous IgG4-positive plasma cells (100-120/hpf).
- The meningothelial component was confirmed with epithelial membrane antigen (EMA) immunostaining, while serum IgG4 levels were normal.
Findings:
- The histological features, particularly the abundance of IgG4-positive plasma cells and fibrosis, suggest a potential overlap with IgG4-related disease.
- Despite normal serum IgG4 levels, the inflammatory infiltrate raises suspicion for an association.
Implications:
- This case highlights the diagnostic challenge posed by LP meningioma due to its inflammatory appearance.
- The findings support the hypothesis of an association between LP meningioma and IgG4-related disease, although further research is needed for confirmation.
- Understanding this potential link may refine diagnostic criteria and treatment strategies for this rare meningioma subtype.
Abstract:
Lymphoplasmacyte-rich meningioma (LP meningioma) is a rare form of grade 1 meningioma. It shows dense lymphoplasmacytic infiltrate, mimicking an inflammatory lesion. We present a case of Lymphoplasmacyte-rich (LP) meningioma in the left parasagittal region in a 47-year-old female. On histological examination, it showed dense lymphoplasmacytic infiltrate masking the meningothelial component. There was dense fibrosis and numerous IgG4-positive plasma cells (100-120/hpf), admixed with lymphocytes and few histiocytes. The meningothelial component was highlighted by epithelial membrane antigen (EMA) immunostain. The patient had normal serum IgG4 level. This case highlights the morphological overlap between LP meningioma and IgG4-related disease. The presence of fibrosis and increased IgG4-positive plasma cells as a major inflammatory component in LP meningioma, as demonstrated in the present case and some other previous studies raise suspicion of its association with IgG4-related disease. However, this hypothesis requires further detailed studies for confirmation.
