Metastatic CNS lymphoma presenting with periventricular dissemination — MRI and neuropathological findings in an autopsy case

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Abstract

Metastatic CNS lymphoma usually manifests as pachymeningeal or leptomeningeal infiltrates, and periventricular dissemination is rare. A 70-year old man first noticed a mass in the left supraclavicular fossa, and then presented with bilateral parkinsonism, followed by consciousness disturbance. Fluid attenuated inversion recovery (FLAIR) image of brain MRI demonstrated hyperintensities at the parenchyma around the lateral ventricle, third ventricle, and fourth ventricle. Gadolinium-enhanced T1-weighted image demonstrated enhancement along the whole wall of the ventricle. Biopsy of the left supraclavicular lymph nodes established a diagnosis of diffuse large B-cell lymphoma. The patient died of multiple organ failure about 5 months after the onset. Autopsy disclosed periventricular dissemination of lymphoma cells that was most severe around the lateral ventricle. We considered that the lymphoma cells entered the ventricular system through the choroid plexus of the lateral ventricle, followed by dissemination of the periventricular parenchyma.

Introduction

In 5–9% of systemic non-Hodgkin's lymphoma, secondary involvement is seen in the CNS, and usually manifests as pachymeningeal or leptomeningeal infiltrates [1], [2]. Parenchymal lesions, when present, typically result from secondary involvement from the leptomeninges [1]. Here, we report an autopsy case of metastatic CNS lymphoma showing an unusual distribution.

Section snippets

Case report

A 70-year-old man had noticed a mass in the left supraclavicular fossa, but did not consult a hospital. About 1 month later, he presented with bilateral parkinsonism including gait disturbance, akinesia, mask-like face, and dysphagia. He was admitted to our hospital about 3 months after the onset. Physical examination on admission demonstrated consciousness disturbance with Glasgow Coma Scale (GCS) of 12 (E3 V4 M5), swelling of the left supraclavicular lymph nodes, measured approximately 4 by

Discussion

We demonstrated periventricular involvement on MRI, and periventricular dissemination of lymphoma cells on postmortem study in a patient with systemic lymphoma. The enhancement with contrast medium is usually due to a breakdown of the blood-brain barrier (BBB). In our case, periventricular lesions included vascular proliferation probably associated with tumor neovascularity. Because the abnormal vessels had no effective BBB, the periventricular areas were distinctly enhanced by contrast medium.

Acknowledgement

This work was supported by a grant-in-aid for scientific research from the Ministry of Education, Culture, Sports, Science and Technology (14570957) and a research grant from the Zikei Institute of Psychiatry.

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