Abstract Details

Title Characterization of Clinical Course, Diagnosis, Treatment, and Outcomes of Intravascular Lymphoma with Neurologic Involvement

Topic Neuro-oncology

Presentation(s) P2 - Poster Session 2 (2:45 PM-3:45 PM)

Poster/Presentation
Number 099

Objective Are patients with IVL causing CES diagnosed sooner if they have rash or constitutional symptoms?

Background Intravascular lymphoma (IVL) is a rare, treatable lymphoma that causes end-organ damage via vascular occlusion. IVL rarely presents as cauda equina syndrome (CES), which can mimic a neuroinflammatory disorder, delaying diagnosis.

Design/Methods Our institution's pathology database and Pubmed and Embase were reviewed from January 1979 to June 2024. Inclusion criteria were biopsy/autopsy confirmed IVL and CES as initial neurological presentation. Exclusion criteria were absence of biopsy/autopsy confirmation, lack of CES as initial presentation, or age less than 18.

Results 2 subjects and 17 case reports were identified. The average age was 59 (range 38-86). 0/4 had rash at initial presentation; 4/5 had rash later. 7/12 had constitutional symptoms at initial presentation; 10/12 had constitutional symptoms later. The most frequent biopsy sites were bone marrow (12), nerve (6) and skin (5); the most frequent confirmation sites were nerve (5), brain (5) and skin (4). 6/14 patients had normal CSF; 9/14 had increased cellularity (average 17 cells/mcL, range 4-36). 0/14 had malignant findings on CSF cytology. 8/15 patients had cauda equina enhancement on MRI initially, increasing to 12/14 throughout disease course. 3/19 patients passed away before being diagnosed. The median time from presentation to diagnosis was 60 days (range 15-690).

Conclusions Rash and constitutional symptoms may not be present at onset of CES, potentially limiting their ability as clues for rapid diagnosis. However, vigilance for these symptoms may still be helpful, as many developed them later. A similar pattern with cauda equina enhancement was observed. Skin biopsy may be an underutilized tool; it was performed on 21% of patients, yielding diagnosis in all with rash. Our study had several limitations including retrospective design, small sample, and heterogeneous data reporting from case reports.

Authors/Disclosures
Lucas Horta, MD PRESENTER	Dr. Horta has nothing to disclose.
Bruna Leles Vieira de Souza, MD (Work)	Miss Leles Vieira de Souza has nothing to disclose.
Denis T. Balaban, MD (Massachusetts General Hospital)	Dr. Balaban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Infucare. The institution of Dr. Balaban has received research support from ArgenX.
Maria Martinez-Lage, MD (Massachusetts General Hospital)	The institution of Dr. Martinez-Lage has received research support from NIH.
Deborah Forst, MD	Dr. Forst has stock in Eli Lilly. Dr. Forst has received research support from Conquer Cancer Foundation of the American Society of Clinical Oncology. Dr. Forst has received research support from Palliative Care Research Cooperative Group. Dr. Forst has received research support from American Cancer Society Institutional Research Grant.

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