title: Recent developments and controversies in primary central nervous system lymphoma.
abstract: OBJECTIVE
  Primary central nervous system lymphoma (PCNSL) is a rare tumor, and its optimal management is a matter of controversy. Recent developments of collaborative groups devoted to PCNSL have allowed to conduct randomized trials addressing important issues. In addition, genome wide analysis provided new insights in the PCNSL tumorigenesis.
  
  
  RESULTS
  The purpose of the present review is to focus on the most recent findings in the management and the biological advances of PCNSL.
  
  
  CONCLUSIONS
  Cumulating data challenge the role of consolidation radiotherapy after high-dose methotrexate-based polychemotherapy, especially in the elderly, in order to reduce delayed neurotoxicity, support intensive chemotherapy with autologous stem cell transplantation as a therapeutic option in PCNSL, confirm the interest for adding rituximab in the initial treatment, and suggest the importance of B-cell receptor/Toll-like receptor/nuclear factor kappa B signaling pathway activation in PCNSL as a promising target for novel therapies.
authors.affiliation: aDepartment of Clinical Neurosciences and Department of Oncology, Centre hospitalier universitaire Vaudois, University of Lausanne, Lausanne, Switzerland bAPHP, Department of Neurology Mazarin, Hôpital Pitié-Salpêtrière, Sorbonne Universités, UPMC, IHU, ICM, LOC Network, Paris, France.
authors.forename: Andreas F
authors.initials: AF
authors.lastname: Hottinger
authors.forename: Agusti
authors.initials: A
authors.lastname: Alentorn
authors.forename: Khê
authors.initials: K
authors.lastname: Hoang-Xuan
mesh_terms: D016543:Central Nervous System Neoplasms / Q000175:diagnosis / Q000628:therapy*; D003131:Combined Modality Therapy; D060830:Consolidation Chemotherapy / Q000379:methods; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007167:Immunotherapy / Q000379:methods*; D058990:Molecular Targeted Therapy / Q000379:methods*; D033581:Stem Cell Transplantation / Q000379:methods; D014182:Transplantation, Autologous
chemical_list: D007155:Immunologic Factors