Immunodeficiency-associated CNS lymphomas

A.Prof Frank Gaillard et al.

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

Pathology

In most instances, and unlike sporadic primary CNS lymphomas, these tumours 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 tumours are more likely to be: 

  • heterogeneous including central non-enhancement (necrosis)
  • multifocal
  • surrounded by a greater degree vasogenic oedema
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Article information

rID: 53116
Synonyms or Alternate Spellings:

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