title: Advances in Primary Central Nervous System Lymphoma.
abstract: Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that is limited to the CNS. Although novel imaging techniques aid in discriminating lymphoma from other brain tumors, definitive diagnosis requires brain biopsy, vitreoretinal biopsy, or cerebrospinal fluid analysis. Survival rates in clinical studies have improved over the past 20 years due to the addition of high-dose methotrexate-based chemotherapy regimens to whole-brain radiotherapy. Long-term survival, however, is complicated by clinically devastating delayed neurotoxicity. Newer regimens are attempting to reduce or eliminate radiotherapy from first-line treatment with chemotherapy dose intensification. Significant advances have also been made in the fields of pathobiology and treatment, with more targeted treatments on the horizon. The rarity of the disease makes conducting of prospective clinical trials challenging, requiring collaborative efforts between institutions. This review highlights recent advances in the biology, detection, and treatment of PCNSL in immunocompetent patients.
authors.affiliation: Department of Neurology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 356, Rochester, NY, 14642, USA. Lauren_Patrick@urmc.rochester.edu.
authors.forename: Lauren B
authors.initials: LB
authors.lastname: Patrick
authors.affiliation: Department of Neurology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 356, Rochester, NY, 14642, USA. nimish_mohile@urmc.rochester.edu.|Department of Neurology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 704, Rochester, NY, 14642, USA. nimish_mohile@urmc.rochester.edu.
authors.forename: Nimish A
authors.initials: NA
authors.lastname: Mohile
mesh_terms: D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols; D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D060830:Consolidation Chemotherapy / Q000379:methods; D006801:Humans; D060828:Induction Chemotherapy / Q000379:methods; D008223:Lymphoma / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D008279:Magnetic Resonance Imaging / Q000379:methods; D016879:Salvage Therapy / Q000379:methods
chemical_list: D000970:Antineoplastic Agents
keywords: Bone marrow transplant; Diffuse large B cell lymphoma; High-dose methotrexate; Leptomeningeal metastases; Leukoencephalopathy; Ocular lymphoma; Primary central nervous system lymphoma