PCNS lymphoma - with hemorrhage

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Weakness.

Patient Data

Age: 75 years
Gender: Female

Pre and post contrast CT images demonstrate two dominant hyperdense and enhancing lesions; one in the rostral midbrain and the other involving the splenium of the corpus callosum. The former is homogeneous in density and surrounded by moderate surrounding vasogenic edema. The latter demonstrates hemorrhage. 

Conclusion: 

Distribution and appearance of the non-hemorrhagic lesion strongly favors CNS lymphoma. Hemorrhage in PCNSL is very uncommon, seen more frequently in immunocompromised individuals. 

 

Case Discussion

The patient went on to have surgery.

Histology

MICROSCOPIC DESCRIPTION: 1&2: Paraffin sections show fragments of cerebral white matter. These are densely infiltrated by a population of atypical large mononuclear cells with round and oval vesicular nuclei, many with conspicuous nucleoli and a variable amount of basophilic cytoplasm. These are arranged in diffuse sheets and are seen to infiltrate the walls of small caliber blood vessels. Scattered mitotic figures are noted. There is prominent reactive astrocytic gliosis in adjacent white matter. Immunohistochemistry shows the atypical cells are CD20+, MUM1+, bcl-2+, bcl-6+, CD23-, CD3-, cyclin D1-, CD10-, EBV-. About 95% of the tumor cells are c-myc immunostain positive. About 50% of the cells are p53 positive.

FINAL DIAGNOSIS: non Hodgkin's large B cell CNS lymphoma - non germinal center phenotype.

Discussion

Although hemorrhage in PCNSL is very uncommon, seen more frequently in immunocompromised individuals, the non-hemorrhagic lesion in this case, and the distribution still makes lymphoma the most likely diagnosis. Multicentric high grade glioma would be an alternative ddx. 

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