Title : Intraocular lymphomas associated with primary CNS lymphomas: Great mystification. Clinical landscape and diagnostic challenges
Abstract:
Primary intraocular lymphomas (PIOL) or vitreoretinal lymphomas (VRL) are a type of primary central nervous system lymphoma (PCNSL), accounting for approximately 2–3% of all non-Hodgkin lymphomas and developing in about 11–25% of patients with PCNSL. Morphologically, PIOLs are diffuse large B-cell lymphomas in more than 90% of cases. PIOLs are characterized in the vast majority of cases by involvement of the vitreous, retina, and/or, less rarely, the optic nerve.
The diagnosis of PIOL is very challenging and in the vast majority of cases patients get the right diagnosis months or years after the onset of clinical manifestations as clinical picture is non-specific and PIOL can mimic other ocular diseases. Patients present to an ophthalmologist with complaints of blurred vision (40-50%), decreased visual acuity (25-30%), and floaters (20-25%). Given the low awareness of possible clinical manifestations of the disease, ophthalmologists most often mistake PIOL symptoms for uveitis or posterior vitreous detachment. Consequently, establishing the correct diagnosis takes an average of 6 to 40 months from the onset of clinical symptoms. For comparison, the average time to diagnosis for PCNSL is 35 days.
The process is often binocular and is accompanied by decreased visual acuity, corneal precipitates, vitreous lymphoid infiltration and subretinal infiltrates. In the advanced stage PIOL can lead to tractional retinal detachment.
The pathogenesis of intraocular involvement in PCNSL is not fully understood. Specific somatic mutations play an important role, determining tumor biology and serving as potential diagnostic markers. The most common mutations are found in the MYD88 and CD79B genes.
The main principles of therapy include intravitreal administration of chemotherapeutic agents (methotrexate, rituximab) and stereotactic radiotherapy (SRT) to the area of the affected eye.
We hereby present a clinical case of a patient with PIOL.
Clinical Case: Patient 1. Female, 55 y/o. Presented to Burdenko National Medical Research Center for Neurosurgery with walking difficulties and memory troubles in may 2022. After total examination and biopsy she was diagnosed with PCNSL , diffuse large B-cell type. The ophthalmic examination revealed decreased vision of the left eye, accompanied with severe redness, corneal (mutton –fat) precipitates and vitreal cloud-like haze. Two years ago the patient the first time experienced these symptoms and was diagnosed with uveitis. Local steroids and immunosuppressive therapy had very slight positive effect. She was tested for HLA-B27, ACE, IGRA, Toxoplasma gondii IgM, HLA-B51, ANA with all negative results. She had radiotherapy at a dose of 20–25 Gy fractionated in 15 sessions for whole brain and left eye with use of lens shielding. Since then she comes for control visits every 3 month revealing no intraocular or brain relapse.
