Intravascular CNS lymphoma

Case contributed by A.Prof Frank Gaillard

Presentation

6 weeks of progressive ataxia, falls, 2 weeks of cognitive impairment.

Patient Data

Age: 66 years
Gender: Male
MRI

Extensive deep white matter signal change demonstrated on both FLAIR and T2. The signal change extends to the subcortical region and also involves the splenium of the corpus callosum. The signal change appears more extensive posteriorly. No contrast enhancement of these areas or abnormal enhancement elsewhere. The posterior fossa appears uninvolved.

The time of flight and post contrast MRA (not shown) demonstrate normal anatomical variance with a foetal origin of the right PCA. There is no evidence of stenosis, or mural enhancement of the circle of Willis vasculature to suggest vasculitis.

CONCLUSION:
There is extensive bilateral white matter FLAIR signal change which is increased since the previous examination. The differential diagnosis includes demyelination (including ADEM) despite the patient's age. Other possibilities include vasculitis or intravascular lymphoma. 

Case Discussion

The patient went on to have a brain biopsy (right frontal lobe) 

Histology

MICROSCOPIC DESCRIPTION
The sections  of  cerebral  white  matter are  mildly  hypercellular,  with focally prominent perivascular  lymphocytic cuffing.   There are diffusely increased astrocytes  through the tissue, as well  as a light scattering of macrophages and  activation of microglial  cells.  The  lymphocytes cuffing vessels are predominantly small CD3-positive T cells.  Within some of these vessels are large atypical  lymphoid cells with a  high proliferative index on the Ki67 stain.   Occasional single large atypical  B-cells are present within  perivascular   parenchyma.   The  features  are consistent  with a diagnosis of intravascular large B cell lymphoma.
 
DIAGNOSIS

Right frontal white  matter biopsies  - large B-cell  non-Hodgkin lymphoma, consistent with intravascular large B-cell lymphoma.

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Case information

rID: 37944
Case created: 26th Jun 2015
Last edited: 10th Sep 2015
Inclusion in quiz mode: Included

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