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Lymphoplasmacyte-rich meningioma mimicking idiopathic hypertrophic pachymeningitis

Parima Hirunwiwatkul1, Jonathan D Trobe, Mila Blaivas

  • 1Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan, USA.

Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society
|June 6, 2007
PubMed

Insights

A rare lymphoplasmacyte-rich meningioma (LRM) presented as extensive meningeal thickening, mimicking idiopathic hypertrophic pachymeningitis (IHP). Early differentiation is crucial for appropriate treatment of this uncommon brain tumor.

Area of Science:

  • Neurology
  • Neurosurgery
  • Pathology

Background:

  • Idiopathic hypertrophic pachymeningitis (IHP) and lymphoplasmacyte-rich meningioma (LRM) are rare conditions that can present with similar symptoms.
  • Distinguishing between IHP and LRM is critical due to their different treatment modalities.

Observation:

  • A 24-year-old male presented with chronic headache and progressive vision loss.
  • Neuro-ophthalmic examination revealed significant visual impairment, pupillary abnormalities, optic disc pallor, and hyperreflexia.
  • Brain MRI demonstrated diffuse meningeal thickening encasing the optic nerves, chiasm, and brainstem, initially suggesting IHP.

Findings:

  • Anterior temporal brain biopsy revealed a lymphoplasmacyte-rich meningioma (LRM), characterized by meningothelial cells and inflammatory infiltrates.
  • The patient's LRM exhibited an unusually extensive mass compared to previously reported cases.
  • Histopathological analysis confirmed LRM, differentiating it from IHP.

Implications:

  • This case highlights the importance of considering LRM in the differential diagnosis of extensive meningeal thickening, even when symptoms suggest IHP.
  • Accurate diagnosis through biopsy is essential for guiding appropriate therapeutic strategies.
  • Further research into LRM may elucidate its unique pathogenesis and inform treatment protocols for similar rare meningiomas.

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