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

(Left) 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).

(Right) 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

ſt, hemispheric white matter

﬇, and cerebellum st.

(Left) Axial T1 C+ MR in the

same case shows intense,

nearly symmetric

enhancement in the inferior

basal ganglia ſt. Note

numerous punctate enhancing

foci in the putamina st and

thalami ﬇. (Right) Coronal T1

C+ MR in the same case shows

curvilinear enhancement st

following the course of the

penetrating lenticulostriate

arteries. Intravascular

(angiocentric) lymphoma was

found at autopsy.

Brain: Pathology-Based Diagnoses: Neoplasms,

Cysts, and Disorders

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