Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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March 19, 2015
Primary Pachymeningeal (Dural) CNS Lymphoma
- Background:
- Primary pachymeningeal CNS lymphoma is rare and comprises 2.4% of primary CNS lymphoma (PCNSL).
- Systemic lymphoma with secondary dural involvement must be excluded.
- Clinical Information:
- More common in middle-aged females (PCNSL: older male)
- Most frequent histology: Marginal zone B-cell lymphoma, low-grade indolent non-Hodgkin lymphoma (PCNSL: diffuse large B-cell lymphoma)
- Key Diagnostic Features:
- Unifocal or multifocal thickening/enhancement of the dura +/- invasive features (leptomeningeal enhancement, parenchymal infiltration). Solid component may show restricted diffusion from hypercellularity.
- DDx:
- Meningioma-en-plaque
- Dural metastases
- Tuberculosis
- Sarcoidosis
- IgG-4-related meningeal disease
- Idiopathic hypertrophic pachymeningitis
- Rx: Systemic or intrathecal chemotherapy and/or radiation