Primary CNS lymphoma

Case contributed by RMH Neuropathology

Presentation

Not available.

Patient Data

Age: 60-year-old
Gender: Female

CT Brain

Modality: CT

Multiple solid and vivid enhancing brain nodules are identified in both frontal lobes and in the right temporal lobe, involving the cortex and adjacent white matter, associated with extensive vasogenic oedema. There is mass effect with compression over the right lateral ventricle and a slight midline shift to the left. The enhancement pattern shows some central hypoattenuating areas within the big nodules and some peripheral ill-defined softness of the enhancing margins. 

MRI Brain

Modality: MRI

Multiple enhancing intra-axial masses in both cerebral hemispheres. All are located close to the grey-white matter interface. All of the lesions located in the left or right frontal lobe except for the largest, which is only right anterior temporal lobe. This measures 2.4 x 2.3 x 2.7 cm.

There is slightly increased diffusion restriction relative to normal brain parenchyma in most of the lesions.

There is vasogenic oedema surrounding all of the lesions. This is particularly marked in the right anterior temporal mass, where vasogenic oedema involves almost the entire right temporal lobe, and extends into the insula, basal ganglia, right cerebral peduncle, and right midbrain.

There is 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.

Conclusion: Appearance compatible with multiple cerebral metastases including lymphoma. Extensive vasogenic oedema and mass-effect.

Modality: Pathology

MICROSCOPIC DESCRIPTION: 1&2: 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. The features are of non-Hodgkin's diffuse large B cell lymphoma.

DIAGNOSIS: 1&2: Brain tumour: non-Hodgkin's diffuse large B cell lymphoma.

Case Discussion

Primary central nervous system lymphomas (PCNSL), as demonstrated in this case, usually present on CT as hyperdense enhancing mass, with MRI T1 hypointense, T2 iso- to hyperintense, vivid homogeneous enhancement and restricted diffusion.

Typically PCNSL are supratentorial (75-85%) and appear as a mass or multiple masses that are usually in contact with the subarachnoid/ependymal surfaces, however, cortical involvement may also happen.

Enhancement on both CT and MRI is pronounced and usually homogeneous.

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Case Information

rID: 40477
Case created: 22nd Oct 2015
Last edited: 13th Nov 2016
Inclusion in quiz mode: Included

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