Characterize the disease course, imaging/laboratory features and clinical outcomes of pathologically confirmed intravascular lymphoma with central nervous system (CNS) involvement.

Intravascular lymphoma is a rare hematologic malignancy, which commonly presents with CNS and skin involvement. There is an incomplete understanding of the typical clinical course, treatment, and outcomes.

We retrospectively reviewed our institutional experience of patients with pathologically confirmed intravascular lymphoma (October 1998-November 2019, last review October 2023). Clinical and pathology data were collected from the electronic medical record. Clinical images were reviewed by a neuroradiologist. We performed descriptive statistics. Survival analyses were performed using the Kaplan Meier method.

We identified 22 patients with CNS involvement of intravascular lymphoma. The median age at diagnosis was 63 years (range: 39-79) with 46% female. Median follow-up was 2.6 years (range: 0.04-17.3). The most common symptoms at presentation were focal neurologic deficit including stroke (73%), constitutional symptoms (59%), cognitive changes (27%), personality changes (18%). Lactate dehydrogenase (LDH) was elevated in most patients (90%, mean 634 units/L). Abnormalities on brain MRI were found in 79% of patients, relative to abnormalities on spine MRI (27%) or angiogram (40%). The most common imaging findings were T2 FLAIR abnormalities (74%), mass-like enhancement (42%), ischemic infarct (26%), meningeal enhancement (26%), and susceptibility weighted changes (5%). CSF cytology was rarely positive for malignancy (6%). The most common first-line therapies were R-CHOP (38%) and R-CHOP+MTX (33%). Median overall survival was 3.8 years (95% CI: 0.8-NA).  

Patients with CNS involvement of intravascular lymphoma present variably, including with constitutional symptoms and elevated LDH as seen in systemic-only involvement. Abnormalities on brain MRI are common but not necessarily seen. When present, characteristic imaging findings included T2 FLAIR abnormalities and mass-like enhancement. CSF cytology was rarely positive for malignancy. As expected, overall prognosis remains guarded.