title: Treatment strategies in primary vitreoretinal lymphoma: a 17-center European collaborative study.
abstract: OBJECTIVE
  The best treatment option for primary vitreoretinal lymphoma (PVRL) without signs of central nervous system lymphoma (CNSL) involvement determined on magnetic resonance imaging or in cerebrospinal fluid is unknown.
  
  
  OBJECTIVE
  To evaluate the outcomes of treatment regimens used for PVRL in the prevention of subsequent CNSL.
  
  
  METHODS
  A retrospective cohort study was conducted at 17 referral ophthalmologic centers in Europe. We reviewed clinical, laboratory, and imaging data on 78 patients with PVRL who did not have CNSL on presentation between January 1, 1991, and December 31, 2012, with a focus on the incidence of CNS manifestations during the follow-up period.
  
  
  METHODS
  The term extensive treatment was used for various combinations of systemic and intrathecal chemotherapy, whole-brain radiotherapy, and peripheral blood stem cell transplantation. Therapy to prevent CNSL included ocular radiotherapy and/or ocular chemotherapy (group A, 31 patients), extensive systemic treatment (group B, 21 patients), and a combination of ocular and extensive treatment (group C, 23 patients); 3 patients did not receive treatment. A total of 40 patients received systemic chemotherapy.
  
  
  METHODS
  Development of CNSL following the diagnosis of PVRL relative to the use or nonuse of systemic chemotherapy and other treatment regimens.
  
  
  RESULTS
  Overall, CNSL developed in 28 of 78 patients (36%) at a median follow-up of 49 months. Specifically, CNSL developed in 10 of 31 (32%) in group A, 9 of 21 (43%) in group B, and 9 of 23 (39%) in group C. The 5-year cumulative survival rate was lower in patients with CNSL (35% [95% CI, 50% to 86%]) than in patients without CNSL (68% [95% CI, 19% to 51%]; P = .003) and was similar among all treatment groups (P = .10). Adverse systemic effects occurred in 9 of 40 (23%) patients receiving systemic chemotherapy; the most common of these effects was acute renal failure.
  
  
  CONCLUSIONS
  In the present series of patients with isolated PVRL, the use of systemic chemotherapy was not proven to prevent CNSL and was associated with more severe adverse effects compared with local treatment.
authors.affiliation: Department of Ophthalmology, University Medical Centre Utrecht, Utrecht, the Netherlands.
authors.forename: Anjo
authors.initials: A
authors.lastname: Riemens
authors.affiliation: Department of Neuro-Oncology, Erasmus Medical Centre, Rotterdam, the Netherlands.
authors.forename: Jacoline
authors.initials: J
authors.lastname: Bromberg
authors.affiliation: Department of Ophthalmology, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Valerie
authors.initials: V
authors.lastname: Touitou
authors.affiliation: Department of Ophthalmology, Tübingen University Hospital, Tübingen, Germany.
authors.forename: Bianka
authors.initials: B
authors.lastname: Sobolewska
authors.affiliation: Rotterdam Eye Hospital, Rotterdam, the Netherlands.
authors.forename: Tom
authors.initials: T
authors.lastname: Missotten
authors.affiliation: Rotterdam Eye Hospital, Rotterdam, the Netherlands.
authors.forename: Seerp
authors.initials: S
authors.lastname: Baarsma
authors.affiliation: Department of Ophthalmology, Radboud Medical Centre, Nijmegen, the Netherlands.
authors.forename: Carel
authors.initials: C
authors.lastname: Hoyng
authors.affiliation: Department of Ophthalmology, Uveitis Unit, University Hospital of León, León, Spain.
authors.forename: Miguel
authors.initials: M
authors.lastname: Cordero-Coma
authors.affiliation: Moorfields Eye Hospital, London, England.
authors.forename: Oren
authors.initials: O
authors.lastname: Tomkins-Netzer
authors.affiliation: Department of Ophthalmology, Heidelberg University Hospital, Heidelberg, Germany.
authors.forename: Anna
authors.initials: A
authors.lastname: Rozalski
authors.affiliation: Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Ilknur
authors.initials: I
authors.lastname: Tugal-Tutkun
authors.affiliation: Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Lausanne, Switzerland.
authors.forename: Yan
authors.initials: Y
authors.lastname: Guex-Crosier
authors.affiliation: Department of Ophthalmology, University Medical Center, Groningen, the Netherlands13W. J. Kolff Institute, Graduate School of Medical Sciences, University of Groningen, Groningen, the Netherlands.
authors.forename: Leonoor I
authors.initials: LI
authors.lastname: Los
authors.affiliation: Department of Ophthalmology, Leiden University Medical Centre, Leiden, the Netherlands.
authors.forename: Jan Geert
authors.initials: JG
authors.lastname: Bollemeijer
authors.affiliation: Bristol Eye Hospital, Bristol, England.
authors.forename: Andrew
authors.initials: A
authors.lastname: Nolan
authors.affiliation: Bristol Eye Hospital, Bristol, England.
authors.forename: Joya
authors.initials: J
authors.lastname: Pawade
authors.affiliation: Department of Ophthalmology, Centre Hospitalier Universitaire St-Pierre and Brugmann, Université Libre de Bruxelles, Bruxelles, Belgium.
authors.forename: Francois
authors.initials: F
authors.lastname: Willermain
authors.affiliation: Department of Ophthalmology, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Bahram
authors.initials: B
authors.lastname: Bodaghi
authors.affiliation: Department of Ophthalmology, University Medical Centre Utrecht, Utrecht, the Netherlands.
authors.forename: Ninette
authors.initials: N
authors.lastname: ten Dam-van Loon
authors.affiliation: Bristol Eye Hospital, Bristol, England.
authors.forename: Andrew
authors.initials: A
authors.lastname: Dick
authors.affiliation: Department of Ophthalmology, Tübingen University Hospital, Tübingen, Germany.
authors.forename: Manfred
authors.initials: M
authors.lastname: Zierhut
authors.affiliation: Moorfields Eye Hospital, London, England.
authors.forename: Susan
authors.initials: S
authors.lastname: Lightman
authors.affiliation: Department of Ophthalmology, Heidelberg University Hospital, Heidelberg, Germany.
authors.forename: Friederike
authors.initials: F
authors.lastname: Mackensen
authors.affiliation: Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Lausanne, Switzerland.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Moulin
authors.affiliation: Department of Ophthalmology, University Medical Centre, Maastricht, the Netherlands.
authors.forename: Roel
authors.initials: R
authors.lastname: Erckens
authors.affiliation: Department of Ophthalmology, Amsterdam Medical Centre, Amsterdam, the Netherlands.
authors.forename: Barbara
authors.initials: B
authors.lastname: Wensing
authors.affiliation: Department of Ophthalmology, Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: Phuc
authors.initials: P
authors.lastname: le Hoang
authors.affiliation: Department of Hematology, University Medical Centre Utrecht, Utrecht, the Netherlands.
authors.forename: Henk
authors.initials: H
authors.lastname: Lokhorst
authors.affiliation: Department of Ophthalmology, Erasmus Medical Centre, Rotterdam, the Netherlands.
authors.forename: Aniki
authors.initials: A
authors.lastname: Rothova
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002423:Cause of Death / Q000639:trends; D003131:Combined Modality Therapy / Q000592:standards; D018572:Disease-Free Survival; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D019572:Retinal Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D014822:Vitreous Body / Q000473:pathology*