title: New approaches in primary central nervous system lymphoma.
abstract: Primary central nervous system lymphoma (PCNSL) has long been associated with an inferior prognosis compared to other aggressive non-Hodgkin's lymphomas (NHLs). However, during the past 10 years an accumulation of clinical experience has demonstrated that long-term progression-free survival (PFS) can be attained in a major proportion of PCNSL patients who receive dose-intensive consolidation chemotherapy and avoid whole brain radiotherapy. One recent approach that has reproducibly demonstrated efficacy for newly diagnosed PCNSL patients is an immunochemotherapy combination regimen used during induction that consists of methotrexate, temozolomide, and rituximab followed by consolidative infusional etoposide plus high-dose cytarabine (EA), administered in first complete remission (CR). Other high-dose chemotherapy-based consolidative regimens have shown efficacy as well. Our goal in this review is to update principles of diagnosis and management as well as data regarding the molecular pathogenesis of PCNSL, information that may constitute a basis for development of more effective therapies required to make additional advances in this phenotype of aggressive NHL.
authors.affiliation: Division of Hematology/Oncology, University of California, San Francisco, CA 94143, USA.
authors.forename: Eleanor
authors.initials: E
authors.lastname: Fraser
authors.affiliation: UCSF School of Pharmacy, University of California, San Francisco, CA 94143, USA.
authors.forename: Katherine
authors.initials: K
authors.lastname: Gruenberg
authors.affiliation: Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, CA 94143, USA. jamesr@medicine.ucsf.edu.
authors.forename: James L
authors.initials: JL
authors.lastname: Rubenstein
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D016543:Central Nervous System Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D016371:Cranial Irradiation; D018450:Disease Progression; D018572:Disease-Free Survival; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D020360:Neoadjuvant Therapy; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D019635:Neurosurgical Procedures; D011237:Predictive Value of Tests; D018714:Radiotherapy, Adjuvant; D012074:Remission Induction; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
keywords: Non-Hodgkin’s lymphoma (NHL); brain tumors; immunotherapy
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