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Primary central nervous system lymphomas—an update

  • Kurt A. Jellinger
  • Werner Paulus
Guest Editorial

Summary

Primary CNS lymphomas (PCNSL), until recently representing about 1% of all brain tumors, show dramatically increased incidence both in high-risk groups (immunocompromised, AIDS) and in the general population. They are extranodal diffuse non-Hodgkin's lymphomas, the morphology and classification of which are identical to those of systemic lymphomas, although PCNSL show different biological behavior and diagnosis according to the New Working Formulation and updated Kiel classification may be difficult. The majority are large B cell variants of high-grade malignancy; lowgrade subtypes and T cell lymphomas are rare. Sixty per cent occur in the supratentorial space (hemispheres, periventricular) and 12% in the posterior fossa; 30% are multiple (50%–70% in AIDS). PCNSL show a male preponderance with a peak incidence in the 5th–7th decade (3rd–4th in AIDS). The duration of diffuse or focal clinical symptoms averages 1–2 months. Computed tomography and magnetic resonance imaging scans show single or multiple or diffuse, often typical lesions. Diagnosis is achieved by evaluation of stereotactic biopsy material or cerebrospinal fluid cytology using immunocytological markers. Current therapy in immunocompetent patients, radiation plus corticosteroids and pre- or postradiation polychemotherapy, shows response rates of 85% with a median survival of 17–44 months, a prognosis similar to that for glioblastoma. Meningeal PCNSL is treated with intrathecal methotrexate or cytosine arabinoside. Transliquoral seeding of PCNSL is frequent, distant metastases occurring in 6%–8%. Therapy of AIDS-related PCNSL makes use of radiation and corticosteroids, and rarely of chemotherapy. The pathogenesis of PCNSL is unknown, but Epstein-Barr virus may be a contributory factor.

Key words

Primary CNS lymphoma Classification Immunophenotype Radiation therapy Chemotherapy Immunodeficiency AIDS 

Abbreviations

NHL

non-Hodgkin's lymphoma

PCNSL

primary central nervous system lymphoma

AIDS

acquired immunodeficiency syndrome

EBV

Epstein-Barr virus

NWF

New Working Formulation (classification system of the National Cancer Institute)

HIV

human immunodeficiency virus

CT

computed tomography

WBRT

whole-brain radiotherapy

MRI

magnetic resonance imaging

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Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • Kurt A. Jellinger
    • 1
  • Werner Paulus
    • 2
  1. 1.L. Boltzmann Institute of Clinical NeurobiologyLainz HospitalViennaAustria
  2. 2.Department of Pathology (Neuropathology)University of Würzburg School of MedicineWürzburgGermany

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