Intravascular lymphoma: magnetic resonance imaging correlates of disease dynamics within the central nervous system

Abstract

Background: Intravascular lymphoma (IVL) is a rare non-Hodgkin's lymphoma with relative predilection for the central nervous system. In the absence of extraneural manifestations, the disease is not recognised until autopsy in the majority of cases underlining the need for new clinical markers. Methods: This is a retrospective series of five patients with IVL seen at a single institution over three years. An advanced magnetic resonance imaging (MRI) protocol was performed at various time points prior to diagnosis and during treatment. Results: MRI revealed multiple lesions scattered throughout the cerebral hemispheres; the brainstem, cerebellum, and spinal cord were less frequently involved. On initial presentation, hyperintense lesions were seen on diffusion weighted images suggestive of ischaemia in three of four patients in whom the images were obtained at that time point. In four patients lesions were also identifiable as hyperintense areas on fluid attenuated inversion recovery (FLAIR) sequences. Initial contrast enhancement was encountered in three cases. Diffusion weighted imaging lesions either vanished or followed the typical pattern of an ischaemic small vessel stroke with evolution of abnormal FLAIR signal followed by enhancement with gadolinium in the subacute stage and tissue loss in the chronic stage. Diffusion weighted imaging and FLAIR abnormalities proved to be partially reversible, correlating with the response to chemotherapy. Conclusion: We provide the first detailed description of the dynamic pattern of diffusion weighted MRI in IVL. These patterns in combination with systemic findings may facilitate early diagnosis and serve as a new tool to monitor treatment response.

High frequency of neurolymphomatosis as a relapse disease of intravascular large B‐cell lymphoma

“…Among the involved organs, it has been demonstrated that IVL has a predilection for CNS involvement at either presentation or relapse; however, neurologic manifestations of IVL are extremely heterogeneous and can include sensory and motor deficits as well as altered consciousness. Neuroimaging findings vary widely and range from the diffuse involvement of the deep white matter to infarctlike lesions, 23 reflecting the heterogeneity of CNS involvement in terms of true lymphoma infiltration and vascular occlusion. In addition, many patients with IVL have a deteriorated performance status at diagnosis, and the diagnosis of CNS involvement often may be made by observing symptoms alone.…”

Intravascular Large B-Cell Lymphoma

“…These changes are similar to changes consistent with CNS vasculitis, are at least partially reversible, and correlate to some degree with disease course and response to chemotherapy. 18 …”

The answer is skin deep: A case of intravascular large B cell lymphoma presenting as progressive paresis and bilateral abducens nerve palsy

“…[1,2,5] Neuroimaging findings are non-specific and imaging abnormalities are detected in about half of patient with IVLBCL who present with neurologic symptoms. [5,6] CT is generally non-diagnostic, though MRI can show non-specific hyperintensity of white-matter lesions suggestive of small-vessel ischemic disease or demyelination. [1,5,6] Given that IVLBCL typically presents as a disseminated disease, no standard procedure for organ biopsy has been established for this disease.…”