March 19, 2015
A 40-year-old man initially presented with gradually worsening headache. Two months later, he presented with seizure. Body CT imaging and whole-body PET-CT failed to reveal any other suspicious lesion.
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
