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Primary central nervous system lymphoma.

J W Grant1, P G Isaacson

  • 1Department of Histopathology, Addenbrooke's Hospital, Cambridge, U.K.

Brain Pathology (Zurich, Switzerland)
|April 1, 1992
PubMed
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Primary central nervous system lymphomas (PCNSL) are increasing in incidence in both immunocompetent and immunosuppressed individuals. While treatment with radiotherapy and chemotherapy improves survival, the prognosis for PCNSL remains poor.

Area of Science:

  • Neurology
  • Oncology
  • Immunology

Background:

  • Primary central nervous system lymphomas (PCNSL) are rare brain tumors, representing <2% of intracranial neoplasms.
  • PCNSL incidence is rising across diverse age groups and populations, including immunocompetent and immunosuppressed individuals.
  • Risk factors include immunodeficiency states (e.g., AIDS) and transplant recipients, with Epstein-Barr virus implicated in immunosuppressed cases.

Purpose of the Study:

  • To review the epidemiology, clinical presentation, diagnosis, and treatment of primary central nervous system lymphomas.
  • To highlight the diagnostic challenges and prognostic factors associated with PCNSL.
  • To discuss the current understanding of PCNSL etiology and behavior.

Main Methods:

  • Literature review of primary central nervous system lymphomas.

Related Experiment Videos

  • Analysis of clinical, radiological, and pathological features.
  • Evaluation of treatment modalities and outcomes.

    • Main Results:

    • PCNSL incidence is increasing, particularly in immunocompromised populations.
    • Clinical presentation is variable, mimicking other neurological conditions; radiological findings often show hyperdense lesions.
    • While most are B-cell lymphomas, T-cell lymphomas are increasingly recognized; immunohistochemistry and genotypic analysis are crucial for diagnosis.
    • Multifocal involvement of the neuraxis and eyes can occur without systemic disease.
    • Surgery's role is uncertain; combined radiotherapy and chemotherapy improve survival but prognosis remains poor, especially in AIDS patients.

    Conclusions:

    • PCNSL is an uncommon but increasingly recognized malignancy with a challenging diagnosis and poor prognosis.
    • Multidisciplinary approaches involving neuro-oncology, radiation oncology, and hematology are essential for optimal management.
    • Further research is needed to elucidate the etiology and improve therapeutic strategies for PCNSL.