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Biology of Primary Central Nervous System Lymphoma

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Lymphoma and Leukemia of the Nervous System

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Abstract

Insights into the biology of primary central nervous system lymphoma (PCNSL) are essential to facilitate the development of more effective treatment for this uncommon variant of extranodal non-Hodgkin lymphoma (NHL). An accumulating body of evidence suggests that the molecular pathogenesis of PCNSL is distinct from systemic lymphomas of the same histological type. First, 90% of NHL cases that present in the central nervous system (CNS) will, upon staging, prove to be confined to the brain, leptomeninges, optic nerves, and intraocular structures and thus be classified as PCNSL. Over the natural history of the disease, it is rare for PCNSL tumors to recur outside of the brain, underscoring this unique tropism for the CNS. Second, PCNSL tumors are associated with an inferior prognosis compared to other localized extranodal NHL tumors confined to a single extranodal site, such as bone. Third, CNS lymphomas tend to exhibit a heightened responsiveness to high-dose methotrexate-based therapy compared to systemic lymphomas with a concomitant diminished responsiveness to adriamycin-based chemotherapy regimens [1].

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Authors and Affiliations

  1. Division of Hematology/Oncology, Department of Medicine, University of California, San Francisco, CA, 94143-1270, USA

    James L. Rubenstein

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  1. James L. Rubenstein
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Correspondence to James L. Rubenstein .

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Editors and Affiliations

  1. , Stephen E. and Catherine Pappas Center f, Massachusetts General Hospital, Fruit St. 55, Boston, 02114, Massachusetts, USA

    Tracy Batchelor

  2. Dept. Neurology, Memorial Sloan-Kettering Cancer Center, York Ave. 1275, New York, 10021-6007, New York, USA

    Lisa M. DeAngelis

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Rubenstein, J.L. (2012). Biology of Primary Central Nervous System Lymphoma. In: Batchelor, T., DeAngelis, L. (eds) Lymphoma and Leukemia of the Nervous System. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7668-0_5

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