Primary CNS lymphoma

Case contributed by Henry Knipe
Diagnosis certain

Presentation

Left sided weakness and mood disturbance. No past medical history.

Patient Data

Age: 60 years
Gender: Female
ct

Extensive white matter hypodensity bilaterally, but by far more severe on the right. There is midline shift and right uncal herniation. No intracranial hemorrhage. There is the impression of a mass at the right temporal pole and in the right superior frontal gyri. 

ct

Multiple vividly enhancing masses are noted bilaterally. These appear intra-axial and occur at the grey-white junction. The right superior frontal mass has central hypodensity. 

mri

Multiple enhancing intra-axial masses in both cerebral hemispheres. All are located close to the grey-white matter interface. They demonstrate increased diffusion restriction. 

There is extensive vasogenic edema surrounding all of the lesions, most severe around the right temporal mass. Marked mass-effect with global sulcal effacement in the right hemisphere, almost complete effacement of the right lateral ventricle, right uncal herniation, compression of the right cerebral peduncle and midbrain, and 9 mm of midline shift to left.

No abnormal susceptibility related signal loss.

HISTOPATHOLOGY​

MACROSCOPIC DESCRIPTION: "Brain tumor": A flat firm piece of brain tissue 30x22x5mm. Serially transversely sectioned and entirely processed.

MICROSCOPIC DESCRIPTION: Paraffin sections show cerebral cortex and white matter which is densely infiltrated by a population of atypical large lymphoid cells. These have round, oval and angulated vesicular nuclei, many with conspicuous nucleoli and a variable amount of pale cytoplasm and are arranged in solid sheets. Aggregation of these atypical cells around blood vessels with invasion of vessel walls is also noted. Frequent mitotic figures are identified. Scattered large reactive astrocytes are admixed with the atypical lymphoid cells.

By immunohistochemistry, the atypical large lymphoid cells are CD20+, CD3-, PAX5+, MUM-1+, bcl-2+. Reactive GFAP+ astrocytes are also noted. EBER-CISH is negative.

DIAGNOSIS: non-Hodgkin's diffuse large B cell lymphoma.

Case Discussion

The patient proceeded to craniotomy and resection of the largest lesion. Histopathology (see previous report) demonstrated non-Hodgkin diffuse large B cell lymphoma. Subsequent staging CT did not demonstrate any further lymphoma deposits. 

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