The diagnosis and treatment of primary vitreoretinal lymphoma: a review

Abstract

Background: To describe the recent diagnostic and treatment options for the most predominant form of primary vitreoretinal lymphoma (PVRL), namely diffuse large B cell lymphoma. This is mainly based on the experience at the Mayo Clinic as well as a partial review of the literature. MYD88 L265P mutation is seen in about 80% of cases; therefore, a polymerase chain reaction for this mutation helps in making the diagnosis that has been notoriously difficult to make. Local therapy using intravitreal methotrexate and rituximab has been very helpful in the treatment of the local disease. Systemic high-dose intravenous methotrexate is helpful in treating bilateral disease in conjunction with intravitreal therapy. Whether it is helpful in preventing or delaying the development of central nervous system lymphoma (CNSL) is still in dispute. If there is development of CNSL or recurrent ocular disease, alternatives to high-dose methotrexate under investigation include pomalidomide, stem cell transplantation, or ibrutinib, with or without local therapy. Vitrectomy alone might be helpful as a debulking procedure. Because of the risks of redevelopment of disease, local radiation should be given if other options are not possible. Aqueous levels of IL10 are helpful in following the redevelopment of local disease.

Conclusion: Although PVRL is still a difficult disease to diagnose and treat, new advances are helping to make these easier. Larger collaborative studies will be helpful in determining better treatments.

Keywords: Diffuse B cell lymphoma; Ibrutinib; MYD88 L265P; Vitreoretinal lymphoma.

Fig. 1

Diffuse large B cell lymphoma in the vitreous of a patient (a). Cytomorphology of the vitreoretinal diffuse large B cell lymphoma cells (H&E ×400—photograph thanks to Diva Salomao, MD (b). Immunostaining for CD20 of the vitreoretinal lymphoma cells (×400) (c). Ki-67 staining of the vitreoretinal cells; note the extensive staining which is consistent of marked local replication (×200) (d)

Fig. 2

Creamy white retinal involvement by vitreoretinal lymphoma was suspected (a) and a vitrector was used to make the diagnosis (b). Final photograph following biopsy and treatment with intravitreal methotrexate and rituximab therapy and systemic therapy with ibrutinib (c)

Fig. 3

Algorithm for treatment strategies for vitreoretinal lymphoma at Mayo Clinic. MRT methotrexate, rituximab, temozolomide, HD MRT high-dose methotrexate, rituximab, temozolomide, ASCT autologous stem cell transplant