Clinical Experience in a Large Cohort of Patients with Vitreoretinal Lymphoma in a Single Center

Ocul Immunol Inflamm. 2021 Apr 3;29(3):472-478. doi: 10.1080/09273948.2020.1787460. Epub 2020 Aug 26.


Background/aims: To report our five-year experience on vitreoretinal lymphoma (VRL) as a single-center tertiary hospital. Methods: The ophthalmic, cytopathology, and onco-hematologic records of patients with VRL consecutively seen from 2014 to 2019 were reviewed. Results: Fifty-nine eyes of 31 patients with large B-cell VRL were included. Eighty-one percent has developed central nervous system lymphoma at the end of follow-up. Several different imaging findings were noted, including vitritis, leopard spot appearance, Bruch's membrane/RPE infiltrations, and ellipsoid zone disruption. A variable combination of MYD88-L265P mutation in the aqueous and/or in the vitreous and positive cytology/histology allowed to reach a definite diagnosis in all the patients. Therapies included intravitreal injections of methotrexate and rituximab, systemic chemotherapy, pan-encephalic radiotherapy, and hematopoietic stem cell transplantation. Conclusion: No definite guidelines exist for VRL management. It is crucial to collect as much data as possible from tertiary referral hospitals, which suitably manage a conspicuous number of VRL patients.

Keywords: CNS lymphoma; intravitreal chemotherapy; multimodal imaging; oncology; uveitis; vitreoretinal lymphoma.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Antimetabolites, Antineoplastic / therapeutic use
  • Antineoplastic Agents, Immunological / therapeutic use
  • Central Nervous System Neoplasms / diagnosis
  • Central Nervous System Neoplasms / drug therapy
  • Central Nervous System Neoplasms / genetics
  • Central Nervous System Neoplasms / pathology*
  • Female
  • Humans
  • Intraocular Lymphoma / diagnosis
  • Intraocular Lymphoma / drug therapy
  • Intraocular Lymphoma / genetics
  • Intraocular Lymphoma / pathology*
  • Intravitreal Injections
  • Lymphoma, Large B-Cell, Diffuse / diagnosis
  • Lymphoma, Large B-Cell, Diffuse / drug therapy
  • Lymphoma, Large B-Cell, Diffuse / genetics
  • Lymphoma, Large B-Cell, Diffuse / pathology*
  • Lymphoma, Non-Hodgkin / diagnosis
  • Lymphoma, Non-Hodgkin / drug therapy
  • Lymphoma, Non-Hodgkin / genetics
  • Lymphoma, Non-Hodgkin / pathology*
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Mutation, Missense / genetics
  • Myeloid Differentiation Factor 88 / genetics
  • Retinal Neoplasms / diagnosis
  • Retinal Neoplasms / drug therapy
  • Retinal Neoplasms / genetics
  • Retinal Neoplasms / pathology*
  • Rituximab / therapeutic use
  • Vitreous Body / pathology*


  • Antimetabolites, Antineoplastic
  • Antineoplastic Agents, Immunological
  • MYD88 protein, human
  • Myeloid Differentiation Factor 88
  • Rituximab
  • Methotrexate