title: Diagnosis and treatment of primary CNS lymphoma in immunocompetent patients: guidelines from the European Association for Neuro-Oncology.
abstract: The management of primary CNS lymphoma is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the very few controlled studies available. In 2013, the European Association of Neuro-Oncology created a multidisciplinary task force to establish evidence-based guidelines for immunocompetent adults with primary CNS lymphoma. In this Review, we present these guidelines, which provide consensus considerations and recommendations for diagnosis, assessment, staging, and treatment of primary CNS lymphoma. Specifically, we address aspects of care related to surgery, systemic and intrathecal chemotherapy, intensive chemotherapy with autologous stem-cell transplantation, radiotherapy, intraocular manifestations, and management of elderly patients. The guidelines should aid clinicians in their daily practice and decision making, and serve as a basis for future investigations in neuro-oncology.
authors.affiliation: Assistance Publique des Hôpitaux de Paris, Department of Neurology Mazarin, Hôpital Pitié-Salpêtrière, Université Pierre et Marie Curie, Institut Hospitalo-Universitaire, Institut du Cerveau et de la Moelle, Inserm Unit 1125, UMR Unit 7225, Lymphome Oculo Cerebral Network, Paris, France. Electronic address: khe.hoang-xuan@psl.aphp.fr.
authors.forename: Khê
authors.initials: K
authors.lastname: Hoang-Xuan
authors.affiliation: Department of Clinical Oncology, Nottingham City Hospital, Nottingham, UK.
authors.forename: Eric
authors.initials: E
authors.lastname: Bessell
authors.affiliation: Department of Neuro-Oncology, Erasmus MC Cancer Institute, University Medical Center, Rotterdam, Netherlands.
authors.forename: Jacoline
authors.initials: J
authors.lastname: Bromberg
authors.affiliation: Departments of Neurology and Oncology, Centre Hospitalier Universitaire Vaudois and University of Lausanne, Lausanne, Switzerland.
authors.forename: Andreas F
authors.initials: AF
authors.lastname: Hottinger
authors.affiliation: Department of Medicine I and Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria.
authors.forename: Matthias
authors.initials: M
authors.lastname: Preusser
authors.affiliation: Department of Neuro-Oncology, University of Turin and City of Health and Science Hospital, Turin, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Rudà
authors.affiliation: Department of Neurology, University Hospital Bochum, Knappschaftskrankenhaus, Bochum, Germany.
authors.forename: Uwe
authors.initials: U
authors.lastname: Schlegel
authors.affiliation: Center for Neuro-Oncology, Davidoff Institute of Oncology, Rabin Medical Center, Campus Beilinson, Petach Tikva, Israel.
authors.forename: Tali
authors.initials: T
authors.lastname: Siegal
authors.affiliation: Hematology Department, Hôpital René Huguenin-Institut Curie, Saint-Cloud, Paris, France; Collège de France, CNRS UMR 7241/INSERM U1050, Paris, France.
authors.forename: Carole
authors.initials: C
authors.lastname: Soussain
authors.affiliation: Radiation Oncology Department, Neolife Medical Center, Besiktas, Istanbul, Turkey.
authors.forename: Ufuk
authors.initials: U
authors.lastname: Abacioglu
authors.affiliation: Department of Ophthalmology, Institut Curie, Paris, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Cassoux
authors.affiliation: Department of Neuropathology, University of Cologne, Cologne, Germany.
authors.forename: Martina
authors.initials: M
authors.lastname: Deckert
authors.affiliation: Department of Neurosurgery, Brain Tumor Center, Erasmus MC Cancer Institute, Rotterdam, Netherlands.
authors.forename: Clemens M F
authors.initials: CM
authors.lastname: Dirven
authors.affiliation: Unit of Lymphoid Malignancies and Division of Onco-hematological Medicine, Department of Onco-hematology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
authors.forename: Andrés J M
authors.initials: AJ
authors.lastname: Ferreri
authors.affiliation: Department of Neurology, Hospital Clinic, Barcelona, Spain.
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.affiliation: Regional Cancer Center Stockholm County, Stockholm, Sweden and Department of Radiation Sciences & Oncology, Umeå University, Umea, Sweden.
authors.forename: Roger
authors.initials: R
authors.lastname: Henriksson
authors.affiliation: Division of Clinical Neuro-Oncology, Department of Neurology, University of Bonn Medical Center, Bonn, Germany.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Herrlinger
authors.affiliation: Department of Neurology, Medical Center Haaglanden, The Hague, Netherlands; Department of Neurology, VU University Medical Centre, Amsterdam, Netherlands.
authors.forename: Martin
authors.initials: M
authors.lastname: Taphoorn
authors.affiliation: Department of Neuro-Oncology, University of Turin and City of Health and Science Hospital, Turin, Italy.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Soffietti
authors.affiliation: Department of Neurology, University Hospital Zürich, Zürich, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Weller
mesh_terms: D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D005060:Europe; D019317:Evidence-Based Medicine; D005260:Female; D006801:Humans; D007121:Immunocompetence; D008223:Lymphoma / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality / Q000628:therapy*; D008297:Male; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D019635:Neurosurgical Procedures / Q000379:methods; D017410:Practice Guidelines as Topic; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012955:Societies, Medical / Q000592:standards; D033581:Stem Cell Transplantation / Q000379:methods; D016019:Survival Analysis; D014182:Transplantation, Autologous / Q000379:methods