Case 01-2017—Primary vitreoretinal lymphoma (PVRL): report of a case and update of literature from 1942 to 2016

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.

Targeting Bruton Tyrosine Kinase With Zanubrutinib for Treatment of Vitreoretinal Lymphoma: Report of 3 Cases

“…Intravitreal methotrexate, as the most common treatment option for VRL, has been shown to effectively control tumor with a total of 25 injections. Intraocular chemotherapy avoids the serious systemic side effects, but it commonly leads to ocular toxicity including corneal epitheliopathy, cataract and ocular hypertension (1). Moreover, it is inconvenient for both patients and physicians, because intraocular chemotherapy has to be done in the ward, and many patients were unable to be admitted inpatient during the pandemic of COVID-19.…”

“…Vitreoretinal lymphoma (VRL) is a rare intraocular malignancy where the lymphocytic neoplastic cells affect mainly in the vitreous and/or retina. It is regarded as a part of the primary central nervous system lymphoma (PCNSL) and shows a close relationship with CNS progression, with a median progression-free survival of 3.5 months in the PCNSL patients with VRL (vs. 8.3 months in those without VRL) (1,2). Currently, no standard treatment approaches have been defined yet, although intravitreal chemotherapy using methotrexate combined with systemic chemotherapy are generally used in the treatment of VRL.…”

Vitreoretinal Lymphoma

“…According to Wang et al [139], PVRL patients with one eye involvement can be treated with local ocular therapy which includes external beam radiotherapy and/or intravitreal chemotherapy with methotrexate (MTX) and/or rituximab (anti-CD20 monoclonal antibody). There is no established treatment protocol for PVRL patients with bilateral involvement.…”

“…Radiation to the eye is a traditional therapy [141,142] and is still used even today for the therapy of PVRL [43,[143][144][145]. In fact, radiation to the eye is highly effective in achieving complete remission of ocular lymphoma with doses ranging from 30 to 45 Gy [139]. However, therapy carries the risk of irreversible radiation retinopathy, optic atrophy and cataract [44,139].…”

“…In fact, radiation to the eye is highly effective in achieving complete remission of ocular lymphoma with doses ranging from 30 to 45 Gy [139]. However, therapy carries the risk of irreversible radiation retinopathy, optic atrophy and cataract [44,139]. While ocular radiation can be used as an initial local therapy for PVRL, it should not be repeated for recurrent PVRL due to increased risk of irreversible vision loss [44,139].…”