A 66-year-old woman presented with dizziness and anemia. Brain MRI revealed an intradural lesion with adjacent vasogenic edema in the right frontal and parietal lobes with a dural tail sign and an arc-shaped homogenously enhancing mass in the subcutaneous soft tissue of the frontal region (Figure 1). Differential diagnosis for this lesion included meningioma, glioma, CNS lymphoma, or autoimmune disorders. Total resection of both lesions revealed pathological morphology consistent with lymphoplasmacytic lymphoma (Figure 2). Detection of an L265P mutation in the MYD88 gene in both CSF and bone marrow biopsy confirmed Bing-Neel syndrome, a rare neurological manifestation of Waldenstrom macroglobulinemia (WM).1 The MYD88 gene encodes a vital protein involved in immune system signaling, with 95% of patients with WM exhibiting an L265P variation. The most common imaging findings are leptomeningeal infiltration or parenchymal involvement of the brain.2 Hematological malignancy should be considered when a meningeal lesion is accompanied by an extracranial tumor.

Figure 1 MRI Images of the Brain

(A) T1-weighted and (B) T2-weighted axial images demonstrate a broad basal lesion (arrowheads) with vasogenic edema. Sagittal (C) and coronal (D) postcontrast T1 flair images demonstrate an enhanced intradural lesion with a dural tail sign (arrows) and an arc-shaped homogenously enhancing mass in the subcutaneous soft tissue (dotted arrows).

Figure 2 Pathology Specimen

Hematoxylin-eosin staining at 40× (A) and 200× (B) shows diffuse dural involvement (yellow arrow, A) and perivascular infiltration (red arrows, B) by lymphocytes with plasmacytoid features. (C) 200× immunohistochemical staining shows lymphocytes are positive for CD20 (red arrows).

Author Contributions

R. Ge: drafting/revision of the manuscript for content, including medical writing for content; study concept or design. J. Wang: drafting/revision of the manuscript for content, including medical writing for content. S. Wang: major role in the acquisition of data. J. Li: composed manuscript and figures.

Study Funding

Medical and Health Research Fund Project in Zhejiang Province (NO.2022KY1185); Ningbo Leading Medical & Health Discipline (2022-F30).

Disclosure

The authors report no relevant disclosures. 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.

Submitted and externally peer reviewed. The handling editor was Resident & Fellow Section Deputy Editor Ariel Lyons-Warren, MD, PhD.

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Received April 25, 2023.Accepted in final form August 28, 2023.© 2023 American Academy of Neurology