Treatment strategies in primary vitreoretinal lymphoma: a 17-center European collaborative study

Anjo Riemens, Jacoline Bromberg, Valerie Touitou, Bianka Sobolewska, Tom Missotten, Seerp Baarsma, Carel Hoyng, Miguel Cordero-Coma, Oren Tomkins-Netzer, Anna Rozalski, Ilknur Tugal-Tutkun, Yan Guex-Crosier, Leonoor I Los, Jan Geert Bollemeijer, Andrew Nolan, Joya Pawade, Francois Willermain, Bahram Bodaghi, Ninette ten Dam-van Loon, Andrew DickManfred Zierhut, Susan Lightman, Friederike Mackensen, Alexandre Moulin, Roel Erckens, Barbara Wensing, Phuc le Hoang, Henk Lokhorst, Aniki Rothova

Research output: Contribution to journalArticleResearchpeer-review


IMPORTANCE: 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.

DESIGN, SETTING, AND PARTICIPANTS: 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.

INTERVENTIONS: 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.

MAIN OUTCOMES AND MEASURES: 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 AND RELEVANCE: 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.

Original languageEnglish
Pages (from-to)191-7
Number of pages7
JournalJAMA Ophthalmology
Issue number2
Publication statusPublished - Feb 2015


  • Adult
  • Aged
  • Aged, 80 and over
  • Biopsy
  • Cause of Death/trends
  • Combined Modality Therapy/standards
  • Disease-Free Survival
  • Europe/epidemiology
  • Female
  • Follow-Up Studies
  • Humans
  • Lymphoma, Non-Hodgkin/diagnosis
  • Male
  • Middle Aged
  • Practice Guidelines as Topic
  • Retinal Neoplasms/diagnosis
  • Retrospective Studies
  • Survival Rate/trends
  • Time Factors
  • Treatment Outcome
  • Vitreous Body/pathology


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