Abstract: Primary vitreoretinal lymphoma (PVRL), as a subset of primary central nervous system lymphoma (PCNSL), is a rare and fatal ocular malignancy. Most PVRL masquerades as chronic posterior uveitis, which makes the clinical diagnosis challenging. Vitreous cells, subretinal lesions and imaging techniques are essential for clinical diagnosis. Importantly, cytopathology/histopathology identification of malignant cells is the gold standard for the diagnosis of PVRL. In addition, molecular detection of immunoglobulin heavy chain (IgH) or T cell receptor (TCR) gene rearrangements, immunophenotyping for cell markers, and cytokine analysis of interleukine-10 elevation are often used as adjunct procedures. Current management of PVRL involves local radiation, intravitreal chemotherapy (methotrexate and rituximab), with or without systemic chemotherapy depending on the involvement of non-ocular tissues. In cases with concomitant PCNSL, systemic high-dose methotrexate/rituximab based therapy in conjunction with local therapy, whole brain radiotherapy and/or autologous stem cell transplantation is considered. Although PVRL normally responds well to initial treatment, high rates of relapse and CNS involvement usually lead to poor prognosis and limited survival. A professional team of medical experts in ophthalmologists, ocular pathologists, neuro-oncologists and hemato-oncologists is essential for optimizing patient management.

Abstract: Acute retinal necrosis (ARN) is a devastating syndrome characterized by panuveitis, retinal necrosis, and a high rate of retinal detachment that may result in poor visual outcomes if not promptly diagnosed and treated. ARN is most commonly caused by viruses within the herpesvirus family. Etiologies include varicella-zoster virus, herpes simplex virus, and cytomegalovirus, and may be promptly diagnosed by polymerase chain reaction testing of aqueous or vitreous fluid. The true incidence of ARN is not known due to its rarity; as a result, clinical treatment is often guided by retrospective case series, case reports, and expert opinion. Standard of care has evolved over time but currently includes a combination of systemic and intravitreal antiviral in conjunction with topical or oral steroids and surgical therapy as needed. Combination therapy may reduce the rate of severe vision loss and increase the rate of visual acuity gain, although further studies are needed in this area. In particular for patients with mild to moderate disease, combination therapy may reduce the rate of retinal detachment. Adjunctive therapies including oral corticosteroid and prophylactic laser barricade are incompletely studied, but corticosteroid in particular, may reduce inflammation, which also is involved in the severe disease pathogenesis observed in ARN. This review discusses the advances in diagnosis and treatment of ARN, including management with combination antiviral medication and surgical interventions.

Background and Objective: Intraocular lymphoma (IOL) is a heterogenous category of rare malignancies that are often misdiagnosed and underrecognized. The rarity of IOL impedes clinical research and contributes to difficulty in standardizing its management. In this article we review the existing scientific literature to identify the current diagnostic tools and discuss comprehensive management of various categories of IOL. Our objective is to increase disease recognition of IOL as a whole and explore updated management options for each subtype.

Methods: PubMed and Embase were searched for publications using the terms ‘intraocular lymphoma’, ‘vitreoretinal lymphoma’, ‘uveal lymphoma’, ‘iris lymphoma’, ‘choroidal lymphoma’ and ‘ciliary body lymphoma’ published from 1990 to June 2021. Inclusion criteria were English language articles. Exclusion criteria were non-English language articles, case reports and animal studies.

Key Content and Findings: IOL often presents in middle-aged and older patients with symptoms of floaters and vision changes, but a broad array of clinical signs and symptoms are possible depending upon subtype. IOL can be subdivided by location of involvement into vitreoretinal and uveal lymphoma. These subtypes express key differences in their pathophysiology, clinical presentation, histology, prognosis, and treatment. Primary vitreoretinal lymphomas (PVRL) generally originate from B-lymphocytes and are associated with central nervous system (CNS) lymphoma. Ophthalmic findings include retinal pigment epithelium changes with yellow subretinal deposits known as “leopard spotting.” Primary uveal lymphomas generally originate from low-grade B-lymphocytes invading the choroid and carry an improved prognosis compared to vitreoretinal lymphomas. Funduscopic findings of primary uveal lymphoma include yellow to pink-yellow choroidal swelling with infiltrative subconjunctival “salmon-patch” lesions. Diagnosis for IOL is often delayed due to insidious onset, low prevalence, and tendency to mimic diseases such as uveitis. Diagnosis may be challenging, often relying on biopsy with specialized laboratory testing for confirmation of IOL. Optimal treatment regimens are currently debated among experts. Management of IOL is best coordinated in association with neuro-oncology clinicians due to the tendency for intracranial involvement.

Conclusions: IOL represents a group of multiple malignancies with distinct clinicopathologic features. Future outlook for treatment and prognosis of IOL is likely to improve with less invasive molecular diagnostic techniques and increased awareness. Clinicians should be circumspect in all patients with possible IOL and promptly refer to oncologic specialists for rapid evaluation and treatment.