title: Pathogenesis and management of primary CNS lymphoma.
abstract: Primary CNS lymphoma (PCNSL), a rare variant of extranodal non-Hodgkin's lymphoma, may cause various neurological symptoms and signs. The best therapeutic strategy is still a matter of debate. High-dose methotrexate (HD-MTX) is the most active compound and should be used as the backbone for any chemotherapy applied. Several other chemotherapeutic drugs have been assessed in combination with HD-MTX, but no standard has yet been defined. Whole-brain radiotherapy is active against PCNSL, but typically does not confer long-lasting remission and is associated with significant neurotoxicity in many patients. The recently published G-PCNSL-SG1 trial has shown that consolidating whole-brain radiotherapy after HD-MTX-based chemotherapy does not prolong overall survival and may therefore be deferred. Combined systemic and intraventricular polychemotherapy, or high-dose chemotherapy followed by stem cell transplantation may offer cures to younger patients. Improving treatment regimens without adding significant (neuro-)toxicity should be the focus of ongoing and future studies.
authors.affiliation: Department of Neurology, University Hospital Zurich, Frauenklinikstrasse 26, Zurich, Switzerland. patrick.roth@usz.ch
authors.forename: Patrick
authors.initials: P
authors.lastname: Roth
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Korfel
authors.forename: Peter
authors.initials: P
authors.lastname: Martus
authors.forename: Michael
authors.initials: M
authors.lastname: Weller
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy