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From the JAMA Network
October 2015

Who Is Who in Primary Vitreoretinal Lymphoma?

Author Affiliations
  • 1Unit of Lymphoid Malignancies, Division of Onco-Hematological Medicine, Department of Onco-Hematology, IRCCS San Raffaele Scientific Institute, Milano, Italy
JAMA Oncol. 2015;1(7):977-978. doi:10.1001/jamaoncol.2015.1223

Intraocular infiltration occurs in 13% of primary central nervous system (CNS) lymphomas (PCNSLs).1 Vitreous, retina, subretinal pigment epithelial space, and optic nerve head are the most commonly affected structures. Ocular involvement can occur as an isolated event, which is currently called primary vitreoretinal lymphoma (PVRL), or can be associated with involvement of other parts of the CNS. Natural behavior and biological and molecular profiles of PVRL are poorly known; mechanisms of lymphomagenesis and homing remain poorly understood, which is mostly due to tumor rarity and to the difficulty of collecting tumor samples suitable for advanced molecular studies. From a clinical standpoint, a variable proportion of patients with PVRL experience CNS dissemination, whereas disease remains confined to the eyes for years in others, even among patients treated with local approaches exclusively (ie, intravitreal chemotherapy, ocular irradiation). As a natural consequence, some investigators reviewed multicenter retrospective series of patients with primary or secondary intraocular lymphoma with the aim to establish the risk of CNS dissemination according to the used treatment.2 In JAMA Ophthalmology, Riemens et al reported a retrospective series of 78 cases of PVRL diagnosed in 17 European ophthalmology units and analyzed the effect of different ocular and systemic treatments both on local disease control and on CNS recurrence prevention. Similarly to previous studies,2 this series displays some selection and interpretation biases; however, the authors should be commended for their effort and methodological scrupulousness, which allow us to discuss relevant issues in this field.

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