title: Primary CNS lymphoma.
abstract: Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system is an aggressive malignancy that exhibits unique biological features and characteristic clinical behaviour, with overall long-term survival rates of around 20-40%. Clinical outcome has improved following the advent of chemoradiation protocols incorporating high-dose methotrexate in the mid-1980s, but disease relapse and adverse neurocognitive sequelae remain major clinical challenges. To address this, investigators have focused on improving drug therapy with novel cytotoxic combinations, monoclonal antibody therapy, and intensive chemotherapy consolidation approaches, in an attempt to improve disease control whilst reducing the requirement for whole-brain radiotherapy. Outcomes for patients that are older, immunocompromised, or have relapsed/refractory disease remain unsatisfactory and there is a paucity of clinical trial data to guide treatment of these groups. This review highlights recent advances in pathobiology, imaging, and clinical management of PCNSL and looks ahead to research priorities for this rare and challenging lymphoid malignancy.
authors: Department of Haematology, Royal Free Hospital, Pond St, London, UK.
authors: Elizabeth H
authors: EH
authors: Phillips
authors: Christopher P
authors: CP
authors: Fox
authors: Kate
authors: K
authors: Cwynarski
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002490:Central Nervous System / Q000187:drug effects / Q000473:pathology* / Q000528:radiation effects; D016543:Central Nervous System Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy*; D006678:HIV / Q000302:isolation & purification; D015658:HIV Infections / Q000150:complications; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D012074:Remission Induction / Q000379:methods; D000069283:Rituximab; D016879:Salvage Therapy / Q000379:methods
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab