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III. Current concepts in primary central nervous lymphoma
Author(s) -
Illerhaus Gerald
Publication year - 2015
Publication title -
hematological oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 44
eISSN - 1099-1069
pISSN - 0278-0232
DOI - 10.1002/hon.2211
Subject(s) - primary central nervous system lymphoma , current (fluid) , primary (astronomy) , medicine , central nervous system , lymphoma , neuroscience , biology , geology , physics , oceanography , astronomy
Primary CNS lymphoma (PCNSL) is a rare disorder defined by involvement of the cerebral parenchyma, leptomeninges, eyes or spinal cord without evidence of systemic disease. PCNSL accounts for approximately 3% of all primary brain tumors and 2–3% of all NonHodgkin’s lymphomas. The incidence of PCNSL rose nearly threefold between 1973 and 1984 with a slight stabilization over the last years. The median age at diagnosis is 61 years rising in patients over 60 years. Approximately 95% of PCNSL is B-cell Non-Hodgkin’s lymphomas classified as diffuse large B-cell lymphomas, whereas indolent B-cell lymphomas and T-cell lymphomas occur rarely. The pathogenesis of PCNSL is controversial. Some concepts indicate that clonal proliferation might occur among normal B lymphocytes drawn to the CNS, a theory supported by the occurrence of white matter brain lesions that herald brain lymphoma. Alternatively, a clone of malignant systemic lymphocytes displaying specific adhesion molecules might travel and penetrate the brain. The International Extranodal Lymphoma Study Group (IELSG) identified the following parameters as independent factors for a poorer outcome: age over 60 years, ECOG performance status greater than 1, elevated serum LDH, high CSF protein concentration, and tumour location within the deep regions of the brain (periventricular regions, basal ganglia, brainstem and/or cerebellum). Patients with 0 to 1, 2 to 3, or 4 to 5 of these adverse risk factors had 2-year overall survival (OS) rates of 80%, 48% or 15%, respectively. A study at the Memorial SloanKettering Cancer Center identified three distinct prognostic classes based on age and performance status only: class 1 (patients<50 years), class 2 (patients≥ 50, Karnofsky performance score≥ 70) and class 3 (patients≥ 50, Karnofsky performance score<70). For diagnostic evaluation, patients should undergo contrast-enhanced brain magnetic resonance imaging and if a lumbar puncture can be performed safely, cytologic evaluation and flow cytometry of CSF. All patients should be subjected to a slit lamp examination as well as CT scans