Primary Malignant Lymphomas of the Central Nervous System in Man
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. Ave
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Primary Malignant Lymphomas of the Central Nervous System in Man K. JELL INGER , TH. RADASKIEWICZ, F. SLOWIK Neurological Institute and Department of Pathology, University of Vienna, and Division of Neuropathology, State Institute of Neurosurgery, Budapest
Summary: Sixty-eight primary malignant lymphomas of the CNS exclusively con-
fined 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 observed. 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 - Immunoblastoma - Immunocytoma - Lymphoblastoma Primary (intrinsic) malignant lymphomas (ML) of the CNS confined exclusively to the brain and its coverings are ranging in incidence from 0.3 to about 1.5 percent of all intracranial neoplasms (cf. 8). Although this group of tumors often referred to as microgliomas/reticulosarcomas (cf. 6) shows an appreciable degree of cytological variations, consistent histological patterns comparable to ML arising in extraneural sites exist and, hence, can be classified according to current taxonomic concepts of non-Hodgkin's lymphomas (4, 5). This paper presents clinico-pathological data and a preliminary classification of 68 primary malignant CNS lymphomas found in a total of about 8000 intracranial neoplasms (incidence 0.85%) MATERIAL AND METHODS Among the intracranial tumors listed during the past 20 years in the files of the Neurological Institute, Vienna, and the State Institute of Neurosurgery,
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K. Jellinger, Th. Radaskiewicz and F. Slowik
Budapest, all primary ML confined e
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