Chapter

Malignant Lymphomas of the Nervous System

Volume 6 of the series Acta Neuropathologica pp 95-102

Primary Malignant Lymphomas of the Central Nervous System in Man

  • K. JellingerAffiliated withNeurological Institute and Department of Pathology, University of ViennaDivision of Neuropathology, State Institute of Neurosurgery
  • , Th. RadaskiewiczAffiliated withNeurological Institute and Department of Pathology, University of ViennaDivision of Neuropathology, State Institute of Neurosurgery
  • , F. SlowikAffiliated withNeurological Institute and Department of Pathology, University of ViennaDivision of Neuropathology, State Institute of Neurosurgery

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Summary

Sixty-eight primary malignant lymphomas of the CNS exclusively confined to the brain and its leptomeninges from a series of about 8000 intracranial neoplasms (incidence 0.85%) were examined and classified according to current histopathologic criteria. Average age at onset of symptoms was 55 years, mean duration of illness to time of diagnosis was 3 months. Survival averaged 1,8 months with supportive care, but 17,2 months with surgery, radiation and/or chemotherapy. CSF cytology was a useful and reliable tool for clinical diagnosis. The cerebral hemispheres were affected in about 50%, the basal ganglia in 18%, posterior fossa in 10%, while multifocal lesions amounted to 22%. All CNS tumors were of the diffuse type of non-Hodgkin’s lymphomas; no follicular (germinal center) lymphomas were observ. d. Three histological patterns comparable to extraneural lymphomas were distinguished: Immunoblastoma (reticulosarcoma) occurred most frequently (58.8%), lympho-plasmacytoid immunocytoma constituted 28 percent, while lymphoblastic lymphoma occurred least frequently (13.2%). There were no significant differences with regard to onset, location, growth pattern or clinical course except for a much poorer prognosis of lymphoblastic lymphoma. Although there are no definite cytological differences between malignant lymphomas arising in extraneural sites or as primary lesions in the CNS, the latter showed a much greater proportion of phagocyting histiocytes (and microglia) and a frequent occurrence of plasmacytes and their precursors which apparently exceeded pure host reaction. The prognostic value of modern classification schemes for CNS lymphomas needs further critical evaluation.

Key words

Malignant Lymphoma Primary CNS Tumor Classification Immuno-blastoma Immunocytoma Lymphoblastoma