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Intravascular (Angiocentric) Lymphoma


KEY FACTS

Terminology

  • Rare malignancy characterized by intravascular proliferation of lymphoid cells with predilection for CNS and skin

Imaging

  • T2/FLAIR multifocal hyperintensities in deep white matter, cortex, and basal ganglia

    • May mimic infarct

  • Diffusion restriction common

  • GRE/SWI: Multifocal hemorrhage often seen

  • Linear and punctate enhancement typical

    • May see meningeal &/or dural enhancement

  • Often mimics vasculitis

Top Differential Diagnoses

  • Vasculitis

  • Multi-infarct dementia

  • Primary CNS lymphoma

  • Neurosarcoid

Pathology

  • Malignant lymphoid cells occlude and distend small arteries, veins, and capillaries

Clinical Issues

  • Dementia is most common presenting feature

  • May present with multiple stroke-like episodes

  • Presents in 5th-7th decades; mean: 60-65 years

  • Diagnosis may be made by skin or brain biopsy

  • Rapidly progressive disease with high mortality rate

    • Mean survival: 7-13 months

  • Rare, but underdiagnosed

    • CNS involved in up to 75% of cases

Diagnostic Checklist

  • Imaging of intravascular lymphoma (IVL) is nonspecific, but IVL should be considered in patients with dementia, multifocal lesions, and enhancement

Malignant lymphoid cells occlude and distend small arteries
, veins, and capillaries, resulting in ischemic lesions
and microbleeds
. Meningeal involvement
is typical of intravascular lymphoma (IVL).

Sagittal T1 C+ MR in a 61-year-old man with known systemic diffuse large B-cell lymphoma and a 1-month history of lethargy and altered mental status shows diffuse linear enhancing foci throughout the basal ganglia
, hemispheric white matter
, and cerebellum
.

Axial T1 C+ MR in the same case shows intense, nearly symmetric enhancement in the inferior basal ganglia
. Note numerous punctate enhancing foci in the putamina
and thalami
.

Coronal T1 C+ MR in the same case shows curvilinear enhancement
following the course of the penetrating lenticulostriate arteries. Intravascular (angiocentric) lymphoma was found at autopsy.

TERMINOLOGY

Abbreviations

  • Intravascular (angiocentric) lymphoma (IVL)

Synonyms

  • Intravascular malignant lymphomatosis

  • Angioendotheliotropic lymphoma

  • Malignant angioendotheliomatosis

  • Angiotropic large cell lymphoma

  • Intravascular B-cell lymphoma

Definitions

  • Rare malignancy characterized by intravascular proliferation of lymphoid cells with predilection for CNS and skin

  • Form of non-Hodgkin lymphoma (NHL) characterized by angiotropic growth

IMAGING

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