Immunodeficiency-associated CNS lymphomas

Last revised by Assoc Prof Frank Gaillard on 21 Oct 2021

Immunodeficiency-associated CNS lymphomas are recognized in the current (2016) WHO classification of CNS tumors as a specific subtype of primary CNS lymphoma. A number of further specific subtypes are recognized, including:


In most instances, and unlike sporadic primary CNS lymphomas, these tumors are associated with prior EBV infection 1. They express EBER1, EBER2, LMP1 and EBNA1-6 1.

Predisposing conditions

Essentially any cause of immunosuppression increases the risk of developing primary CNS lymphoma, including 1

Radiographic features

Although generally, the features of immunodeficiency-associated CNS lymphomas is similar to that of sporadic primary CNS lymphomas, in the setting of immunodeficiency tumors are more likely to be: 

  • heterogeneous peripheral enhancement with central non-enhancement (due to necrosis). This is in contrast to solid homogeneous enhancement in immunocompetent lymphoma
  • multifocal
  • surrounded by a greater degree vasogenic edema

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Cases and figures

  • Figure 1: gross pathology
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  • Case 1
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  • Case 2
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