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Primary central nervous system lymphomas--an update.

K A Jellinger, W Paulus

    Journal of Cancer Research and Clinical Oncology
    |January 1, 1992
    PubMed
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    Primary CNS lymphomas (PCNSL) are increasing in incidence. Current treatments offer a median survival of 17-44 months, similar to glioblastoma.

    Area of Science:

    • Neuro-oncology
    • Hematology
    • Immunology

    Background:

    • Primary CNS lymphomas (PCNSL) are extranodal diffuse non-Hodgkin's lymphomas with increasing incidence in both immunocompromised and general populations.
    • Diagnosis can be challenging due to similarities with systemic lymphomas and evolving classification systems.
    • The majority of PCNSL are high-grade large B-cell lymphomas, with rare low-grade or T-cell subtypes.

    Discussion:

    • PCNSL predominantly affect the supratentorial space and posterior fossa, often presenting as multiple lesions, particularly in AIDS patients.
    • Clinical symptoms typically manifest over 1-2 months, with imaging revealing characteristic lesions.
    • Diagnosis relies on stereotactic biopsy or cerebrospinal fluid cytology with immunocytological markers.

    Key Insights:

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    • Current therapy for immunocompetent patients (radiation, corticosteroids, chemotherapy) yields high response rates (85%) and a median survival of 17-44 months.
    • Meningeal PCNSL requires specific treatment like intrathecal chemotherapy.
    • AIDS-related PCNSL treatment primarily involves radiation and corticosteroids, with chemotherapy used less frequently.

    Outlook:

    • The pathogenesis of PCNSL remains unclear, though Epstein-Barr virus is a potential contributing factor.
    • Further research into PCNSL pathogenesis and treatment optimization is warranted.
    • Understanding PCNSL biology is crucial for improving patient outcomes and developing targeted therapies.