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Images in Neurology |

Diffusely Infiltrating Central Nervous System Lymphoma Involving the Brainstem in an Immune-Competent Patient

Khalid Alsherbini, MD1; Brad Beinlich, MD1; M. Shahriar Salamat, MS, MD, PhD2
[+] Author Affiliations
1Department of Neurology, University of Wisconsin, Madison
2Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison
JAMA Neurol. 2014;71(1):110-111. doi:10.1001/jamaneurol.2013.1578.
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A 49-year-old right-handed woman presented with a 3-week history of progressive ataxia, dysarthria, right hemiparesis, cognitive decline, and headache. Infectious, inflammatory, neoplastic, and paraneoplastic workup results were unremarkable. The ophthalmologic evaluation findings were unremarkable for inflammatory or neoplastic involvement. Repeated lumbar puncture findings revealed mild lymphocytic pleocytosis with mildly elevated proteins. Cerebrospinal fluid cytology and flow cytometry results were negative. Magnetic resonance imaging of the brain showed a diffusely enhancing lesion typical for CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids).1 Biopsy was felt to be risky given the deep location of the lesion, hence the patient was treated with steroids. The patient showed moderate clinical improvement initially and there was significant reduction in edema on magnetic resonance imaging but persistent perivascular curvilinear enhancement (Figure 1 and Figure 2). With subsequent clinical progression while the patient was taking steroids, a cerebellar biopsy was performed and revealed primary large B-cell non-Hodgkin lymphoma (Figure 3). The patient underwent 4 cycles of high-dose methotrexate, rituximab, and temozolomide. Following the first 2 cycles, she had a complete resolution of her speech symptoms and significant improvement of her right-sided weakness. Unfortunately, her treatment was complicated by steroid-induced diabetes mellitus, bilateral avascular necrosis of the hips and right shoulder, and septic right hip arthritis, which resulted in deconditioning and generalized weakness. Magnetic resonance imaging at 6 months showed complete resolution of abnormal enhancement and near-complete resolution of her abnormal T2–fluid-attenuated inversion recovery hyperintense signal abnormality, and the patient was considered to be in remission.

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Figure 1.
Magnetic Resonance Imaging of the Brain

A, T1-postcontrast magnetic resonance coronal sequence showing a contagious enhancing lesion extending from the pons along the white matter tracts to involve the bilateral basal nuclei. B and C, T1-postcontrast magnetic resonance axial sequences showing the curvilinear perivascular enhancement along perforating arterioles of the crus cerebri in the midbrain, pons, and cerebellum, which is worse on the left and typical for CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids).

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Figure 2.
Additional Magnetic Resonance Imaging of the Brain

Magnetic resonance imaging and fluid-attenuated inversion recovery sequences show an abnormal signal along the previously noted enhanced area with minimal vasogenic edema.

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Figure 3.
Histologic Analysis

A, At low magnification, the hematoxylin and eosin–stained section reveals infiltration mostly of the cerebellar white matter by a densely cellular neoplasm (original magnification ×10). Note the cerebellar cortex including portions of the granular, pyriform, and molecular cell layers on the left upper half of the photo. B, At ×40 magnification, the hematoxylin and eosin–stained tumor consists of cells with scant cytoplasm and enlarged nuclei. The nuclei are irregular and vary in size. A mitotic figure is also present (arrowhead). C and D, At ×20 magnification, tumor immunolabeled with CD20 and CD3 antibodies, respectively, are shown. A large number of tumor cells with large nuclei are positively labeled with CD20 antibody, while the adjacent section labeled with CD3 contains a much smaller number of benign T cells with small nuclei.

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