Lymphomatoid granulomatosis (CNS manifestations)

Last revised by Assoc Prof Frank Gaillard on 21 Oct 2021

Lymphomatoid granulomatosis of the central nervous system is uncommon, but represents the second most common site of involvement in patients with systemic lymphomatoid granulomatosis, after the lungs, which are most commonly involved. It is considered one of the immunodeficiency-associated CNS lymphomas under the current (2016) WHO classification of CNS tumors 3

For a general discussion of the underlying condition, including epidemiology and pathology, please refer to the article lymphomatoid granulomatosis

Neurologic symptoms are fairly common, seen in around 30% of cases, usually in the setting on systemic or cutaneous symptoms 1

Like other immunodeficiency-associated CNS lymphomas, lymphomatoid granulomatosis is frequently EBV-associated 3. The lesions composed of infiltrating lymphocytes are typically angiocentric or angiodestructive 3

MRI is the modality of choice for assessing patients with suspected lymphomatoid granulomatosis. 

Appearances are very variable with lesions seen in all compartments (supra- and infratentorial) and demonstrating a wide range of morphology.

Generally, lesions are located in the periventricular white matter but can extend to involve cortex.

Punctate or linear T2 hyperintensities within perivascular spaces are also characteristic and show contrast enhancement 1,2. Larger lesions may be solid or demonstrate ring enhancement. They have variable surrounding edema and may be associated with leptomeningeal enhancement 1

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