A 47-year-old woman presented with a 6-month history of occipital-cervical region pain, numbness of both upper limbs, and progressive bilateral hearing loss. Enhanced MRI revealed a lesion involving the intracranial dura mater and the right trigone area of the lateral ventricle (figure, A–J). Surgery to relieve the main symptoms was performed through a posterior midline approach (figure, K and L). Pathology suggested the diagnosis of lymphoplasmacyte-rich meningioma (figure, M–P). Lymphoplasmacyte-rich meningioma is a rare variant of meningioma and is categorized as a WHO grade I tumor and approximately 60 cases have been reported,1 but involvement of the entire intracranial dura mater is unusual. It is characterized by dense lymphoplasmacytic infiltration and usually treated with surgical resection1; however, total resection could not be achieved in our case. The patient received radiotherapy and postsurgery MRI at 3 months but demonstrated no progress.
MRI: T1-weighted (A), T2-weighted (B), and gadolinium contrast (C) of bilateral cerebellopontine angle (CPA); bilateral cerebellar tentorium (D); bilateral foramen magnum (E); falx cerebri and bilateral frontal base (F); falx cerebri, bilateral parietal lobe, bilateral cerebellar tentorium, and bilateral CPA (G); falx cerebri, bilateral cerebellar tentorium, and the right trigone area of the lateral ventricle (H); frontal base, cerebellar tentorium, and CPA (I); clival region and foramen magnum (J); postsurgery (white arrow) (K); surgery (white arrow) (L); hematoxylin & eosin (magnification, ×200) (M); immunohistology positive: epithelial membrane antigen (magnification, ×200) (N), CD138 (magnification, ×200) (O), immunoglobulin G4 (magnification, ×400) (P).
Author contributions
Xiang Yang: study design, data collection and analysis, drafting the manuscript, revising the manuscript. Jun Le: data collection, drafting the manuscript, revising the manuscript. Xin Hu: acquisition of data, analysis and interpretation of data. Yuekang Zhang: study concept and design. Jiagang Liu: in charge of surgery, clinical care and investigative workup of the patient, study concept and design, and revision of manuscript.
Study funding
No targeted funding reported.
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
- © 2018 American Academy of Neurology
Disputes & Debates: Rapid online correspondence
- Lymphoplasmacyte-rich meningioma mimicking pachymeningitis, but not due to IgG4-related disease
- Lymphoplasmacyte-rich meningioma mimicking pachymeningitis due to IgG4-related disease
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