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].
Keywords
- Primary Central Nervous System Lymphoma
- Central Nervous System Lymphoma
- Primary Central Nervous System Lymphoma Patient
- Systemic DLBCL
- Central Nervous System Microenvironment
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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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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DOI: https://doi.org/10.1007/978-1-4419-7668-0_5
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