An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs

Nozomi Hishikawa, Hisayoshi Niwa, Takashi Hara, Kazuhiro Hara, Makoto Ito, Satoko Shimada, Mari Yoshida, Yoshio Hashizume, Nobuyuki Murakami

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13 Citations (Scopus)

Abstract

We describe the case of a 61-year-old man presenting with subacute encephalopathy. The clinical manifestations included progressive dementia and pyramidal and extrapyramidal tract signs. Brain CT scan and MRI showed diffuse bilateral white matter changes in the cerebral hemispheres, basal ganglia, thalamus and brainstem. No contrast-enhanced lesion was observed. Peripheral blood studies, CSF analysis, and brain and muscle biopsies were nonspecific and failed to reveal diagnostic evidence of any specific disease. The patient was diagnosed with and treated for a cerebral demyelinating disorder. Post mortem examination showed diffuse infiltration of lymphoma cells without mass lesions in the extensive cerebral white and gray matter with minimal intravascular patterns, particularly in the perivascular and periventricular spaces. These findings were consistent with lymphomatosis cerebri (LC). In other visceral organs such as the lungs, liver, kidneys and adrenal glands, blood vessels were plugged by numerous neoplastic cells which were morphologically and immunohistochemically similar to those observed in the CNS, consistent with intravascular malignant lymphoma (IVL). To our knowledge, this is the first autopsy report showing the coexistence of LC and IVL. This case suggests a possible link between LC and IVL.

Original languageEnglish
Pages (from-to)612-619
Number of pages8
JournalNeuropathology
Volume31
Issue number6
DOIs
Publication statusPublished - Dec 1 2011
Externally publishedYes

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Keywords

  • Intravascular malignant lymphoma
  • Large b-cell lymphoma
  • Lymphomatosis cerebri
  • Primary central nervous system lymphoma
  • Progressive dementia

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

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