CNS lymphoma with steroid response

Case contributed by A.Prof Frank Gaillard

Presentation

None provided.

Patient Data

Age: 85 years
Gender: Female

CT Day 0

Modality: CT

The splenium of the corpus callosum and periventricular white matter appears abnormal with positive mass effect and mixed hypo and slightly hyperdensity. A further hyperdensity is seen involving the floor of the fourth ventricle inferiorly. 

MRI Day 1

Modality: MRI

Splenial and brainstem lesions demonstrate vivid contrast enhancement and reduced ADC values. 

Features are characteristic of CNS lymphoma. 

Unfortunately in the interim the patient had received a number of doses of steroids prior to eventually having a biopsy. 

CT Day 6 (post biopsy)

Modality: CT

Non-contrast imaging through the brain has been obtained and compared to preoperative imaging of the brain. Small amount of gas is seen within subarachnoid space anteriorly within the ventricles, and a small locule of gas seen within the right side of the splenium corpus callosum the site of biopsy, which should be within the area of abnormality.

Please note there has been dramatic reduction in the amount of enhancement in the region, presumably due to steroid use; this is further support that this represents CNS lymphoma, however it may make histological assessment more difficult. 

MRI Day 7

Modality: MRI

The solid homogeneousness enhancement along the splenium of corpus callosum and dorsal medulla, with diffusion restriction, is again noted, but is markedly less enhancing than before. Degree to T2 signal change has also reduced. 

These effects are attributable to steroid administration. 

Right parietal occipital burr hole with underlying hemosiderin stained biopsy tract is present, with site of biopsy within the enhancing splenial lesion. 

 

Case Discussion

As expected histological evaluation was difficult due to administration of steroids. 

Histology

MICROSCOPIC DESCRIPTION:

Serial sections show cores of periventricular white matter one of which includes intact ventricular ependymal lining. All show infiltration by CD68+ monocyte-macrophages and there is prominent microglial activation. Moderate numbers of CD3+ T lymphocytes are seen forming narrow cuffs around small calibre blood vessels as well as single cells and small aggregates within intervening parenchyma. No CD20+ lymphoid cells are seen. Numerous corpora amylacea are noted.

There is moderate reactive astrocytic gliosis. No mitotic figures are identified. There is no microvascular proliferation and no necrosis is identified. Myelin appears to be decreased in density but there is no convincing evidence of plaque-like demyelination. No ground-glass oligodendrocytes are seen. There is no immunostaining for IDH-1 R132H or p53. Immunostaining of nuclei for MGMT and ATRX is intact.

Nuclear staining for topoisomerase is seen in approximately 3% of cells. These appear to be monocyte-macrophages and lymphocytes.Immunostaining for SV-40/BKV is NEGATIVE

FINAL DIAGNOSIS: 

Cerebral white matter within normal limits.

Discussion

If a diagnosis is suspected it is important that the patient does not receive steroids as it makes histological proof difficult, even if contrast does not entirely disappear. 

Nonetheless the combination of imaging features, response to steroids and absence of a diagnosis on biopsy are sufficient to make the diagnosis of CNS lymphoma with confidence. 

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Case Information

rID: 37980
Case created: 29th Jun 2015
Last edited: 4th Apr 2017
Inclusion in quiz mode: Included

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