Primary Vitreoretinal Lymphoma – August, 2025
History:
A 82-year-old patient with a past medical history of diffuse large B-cell CNS lymphoma presented with decreased vision in the right eye (OD). He completed 6 cycles of chemotherapy and underwent left side brain radiation 4 months prior to presentation.
Exam:
Visual acuity was 20/150 OD and 20/20 in the left eye (OS). Intraocular pressures were normal. Anterior segment exam was unremarkable aside from pseudophakia. Fundus exam OD revealed deep, yellow, subretinal lesions parafoveally (Fig 1) and Ocular Coherence Tomography demonstrated photoreceptor disruption (Fig 2). Fundus exam OS was unremarkable (Fig 3).
Systemic inflammatory and infectious labs were normal. MRI brain and LP showed remission of CNS lymphoma. The patient was scheduled for a diagnostic vitrectomy but unfortunately had a seizure three nights prior to surgery and received high dose steroid therapy. As a result, diagnostic studies from the vitrectomy were inconclusive for lymphoma. Given his classic presentation, the decision was made to begin treatment with intravitreal methotrexate. He demonstrated significant improvement and eventual resolution of his subretinal lesions (Fig 4) and photoreceptor reconstitution (Fig 5). In conjunction with his neuro-oncologist, systemic chemotherapy was initiated.
Differential Diagnosis:
- Primary vitreoretinal lymphoma
- Secondary intraocular lymphoma
- Infectious or inflammatory uveitis
- Birdshot chorioretinopathy
Discussion:
Primary Vitreoretinal Lymphoma
Primary vitreoretinal lymphoma (PVRL) is a rare, aggressive cancer in older adults where lymphoma cells invade the vitreous and retina.1 It often mimics chronic posterior uveitis and presents as painless, progressive vision changes with yellow subretinal lesions resulting in photoreceptor disruption. Diagnosis entails vitreous or retina sampling to detect lymphoma cells.2,3 PVRL is strongly linked to CNS involvement with most patient developing brain lymphoma. Early diagnosis enables appropriate treatment and vigilant CNS monitoring.4
If you are looking to schedule your first consultation, please contact us today by clicking HERE and find the location that is nearest you!
References:
- Wang SY et al. Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis. Blood Sci. 2025;7:e00233.
- Dalal M, Casady M, Moriarty E, Faia L, Nussenblatt R, Chan CC, Sen HN. Diagnostic procedures in vitreoretinal lymphoma. Ocul Immunol Inflamm. 2014 Aug;22(4):270-6.
- Chan CC, Sen HN. Diagnosing and managing primary vitreoretinal lymphoma. Discov Med. 2013;15:93-100.
- Pulido JS et al. Diagnosis and treatment of primary vitreoretinal lymphoma. Int J Retina Vitreous. 2018;4:18.