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Primary CNS lymphoma

Nature Clinical Practice Neurology volume 3, pages 24–35 (2007)Cite this article

Abstract

Non-Hodgkin's lymphoma invades the brain, the vitreous body and nerves of the eye, the meninges, and the nerve roots of brain and spine, leading to the development of a primary CNS lymphoma. The mechanism of involvement of these locations by malignant B lymphocytes is unknown, but it might involve molecular targeting of lymphoma cells generated at cryptic systemic sites. The diagnosis of primary CNS lymphoma has been facilitated by advances in imaging techniques and the discovery of molecular markers. Methotrexate-based regimens, even when radiation is deferred, prolong overall survival to over 5 years, but relapses eventually occur in most cases. Better tools for earlier diagnosis and monitoring of treatment response will emerge from molecular studies of therapeutic targets.

Key Points

  • Primary CNS lymphoma (PCNSL) is a nervous-system-seeking extranodal non-Hodgkin's lymphoma that shares common morphologic and molecular features with diffuse large B-cell lymphoma

  • Lymphomagenesis is probably promoted through direct transforming properties of infectious agents (Epstein–Barr virus in immunocompromised patients) or sustained antigenic stimulation by infection or autoimmune disease

  • PCNSL presents with syndromes ranging from language deficits, paresis of extremities, brain swelling, brainstem dysfunction, endocrine dysregulation and behavioral abnormalities to peripheral neuropathies and signs of vitreous disease

  • Diagnosis of PCNSL requires histopathological confirmation; clonality assessment by immunoglobulin gene rearrangement analysis or flow cytometry might prove useful not only for initial diagnosis but also for detection of minimal residual disease

  • High-dose methotrexate is an essential component of any treatment regimen for PCNSL

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Figure 1: Neuroimaging findings in patients with primary CNS lymphoma.
Figure 2: The prodrome of primary CNS lymphoma.
Figure 3: Histopathological findings in primary CNS lymphoma.
Figure 4: Analysis of paucicellular specimens.

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Authors and Affiliations

  1. FH Hochberg is Attending Neurologist (neuro-oncology), and EP Hochberg is a medical oncologist and Director of Clinical Lymphoma Research, at the Massachusetts General Hospital Boston, MA 02114, USA,

    Fred H Hochberg & Ephraim P Hochberg

  2. JM Baehring is a neuro-oncologist at Yale University School of Medicine and the Director of the Yale Brain Tumor Center, New Haven, CT, USA.,

    Joachim M Baehring

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Correspondence to Fred H Hochberg.

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Hochberg, F., Baehring, J. & Hochberg, E. Primary CNS lymphoma. Nat Rev Neurol 3, 24–35 (2007). https://doi.org/10.1038/ncpneuro0395

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