Intravascular lymphoma of the central nervous system: a rare subtype of a common disease

A 67-year-old man had a sudden onset of headache and aphasia, evolving to spontaneous improvement. A few months later, he developed dysarthria and left hemiparesis. Brain MRI showed several punctate lesions with perilesional edema, perivascular enhancement and restricted diffusion on DWI ([Figure 1]). PET-CT demonstrated mild uptake ([Figure 1]). Anatomopathological and immunohistochemical analysis were compatible with intravascular large B-cell lymphoma (IVL) ([Figure 2]). Treatment with R-CHOP and intrathecal methotrexate was established, with favorable response due to high tumor sensitivity[1]. Approximately half of IVL cases are diagnosed only after autopsy[2]. The main differential diagnoses are vasculitis, neurosarcoidosis, and ischemic stroke[3],[4].

Figure 1 Magnetic resonance imaging findings of intravascular lymphoma. (A and B) Fluid-attenuated inversion recovery axial images showed diffuse multiple hyperintensities of the cerebral white matter. (C and D) The perivascular curvilinear enhancement on T1-weighted imaging with gadolinium expanded markedly. (E and F) Diffusion-weighted imaging and apparent diffusion coefficient map showed restricted diffusion. (G and H) Perfusion magnetic resonance imaging (relative cerebral blood volume) and positron emission tomography - computed tomography were practically unremarkable.

Figure 2 Brain pathology showing small blood vessels filled with lymphoma cells and perivascular reactive lymphocytes (A and B). Immuno his to chemical staining showing CD20+ (C and D).

Conflict of interest:

There is no conflict of interest to declare.

Authors’ contributions:

RMC, APAF, IGP, SKGM, FFSZ, RCMP, CSC: design or conceptualization of the study, analysis or interpretation of the data, drafting or revising the manuscript for intellectual content.

• References

• 1 Fonkem E, Lok E, Robison D, Gautam S, Wong ET. The natural history of intravascular lymphomatosis. Cancer Med. 2014 Jun;3(4):1010-24. https://doi.org/10.1002/cam4.269
• 2 Domizio P, Hall PA, Cotter F, Amiel S, Tucker J, Besser GM, et al. Angiotropic large cell lymphoma (ALCLL): morphological, immunohistochemical and genotypic studies with analysis of previous reports. Hematol Oncol. 1989 May-Jun;7(3):195-206. https://doi.org/10.1002/hon.2900070303
• 3 Williams RL, Meltzer CC, Smirniotopoulos JG, Fukui MB, Inman M. Cerebral MR imaging in intravascular lymphomatosis. AJNR Am J Neuroradiol. 1998 Mar;19(3):427-31.
• 4 Baehring JM, Henchcliffe C, Ledezma CJ, Fulbright R, Hochberg FH. Intravascular lymphoma: magnetic resonance imaging correlates of disease dynamics within the central nervous system. J Neurol Neurosurg Psychiatr. 2005 Apr;76(4):540-4. https://doi.org/10.1136/jnnp.2003.033662

Publication History

Received: 12 September 2020

Accepted: 19 September 2020

Article published online:
01 June 2023