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primary CNS lymphoma

Monday 9 March 2009

primary lymphomas of the central nervous system

Definition: Primary CNS lymphoma is a rare form of aggressive extranodal non-Hodgkin lymphoma involving the CNS.



They are mainly consisting of highly malignant large CD20-positive B lymphoblasts or centroblasts respectively, and multifocal or diffuse neoplastic infiltration of the brain parenchyma.

The diagnosis typically rests on radiographic features, detection of EBV antigens in the CSF, and histopathology. It is a well-recognized complication in individuals with congenital or acquired immunodeficiency.

The uncontrolled and overwhelming proliferation of B-lymphocytes driven by EBV is believed to be the primary etiological mechanism.

Treatment of primary CNS lymphoma includes high-dose methotrexate polychemotherapy, therapy with the recombinant monoclonal chimeric anti-CD20 monoclonal antibody rituximab, antiviral agents, and whole brain radiation.

Despite aggressive management, the prognosis of EBV-associated primary CNS lymphoma remains extremely guarded with a median survival time of 2-12 months.

Case studies

- [UPMC #600-­>] (EBV-associated diffuse large B cell lymphoma and additional microglial nodule encephalitis of the brainstem with associated severe hypoxic injury of neurons)

See also

- CNS tumors
- Lymphomas