Insights from a Case of Vitreoretinal Lymphoma

Abstract

Purpose/Background: The aim of this study was to report a patient with vitreoretinal lymphoma with clinical features providing hypothesis-generating insights into the pathophysiology of this disease. Methods: Clinical history and imaging studies (i.e., fundus photography, optical coherence tomography, fundus autofluorescence, and fluorescein angiography) were documented. Results: A 71-year-old woman presented with a 2-month history of blurred vision in the right eye and bilateral vitreous infiltrates unresponsive to topical and systemic steroids. Vitreous biopsy of the left eye was diagnostic for lymphoma. Bulky subretinal deposits in the right eye responded to systemic therapy. The left fundus showed diffuse hypoautofluorescence and punctate, hyperfluorescent sub-retinal pigment epithelial tumor deposits, which resolved leaving hypoautofluorescent atrophic retinal pigment epithelium (RPE) scars, except inferotemporally, where retinal vasculopathy had occurred. Conclusions: The clinical features suggest that occlusion of the inferotemporal retinal arteriole prevented sub-RPE lymphomatous deposits and subsequent RPE atrophy in this area of vascular nonperfusion. This suggests that “primary” vitreoretinal lymphoma is secondary to hematogenous spread from systemic loci. This finding, together with the ocular tumor control achieved entirely by systemic therapy, indicates scope for studies investigating systemic treatment protocols, especially those including immune-modulatory agents.

Vitreoretinal lymphoma presenting as frosted branch angiitis in a patient with diffuse large B-cell lymphoma

“… 6 One center favors systemic therapies, reserving intravitreal therapy or vitrectomy for cases with dense vitreous infiltrates. 2 , 18 Others advocate for intravitreal methotrexate and rituximab for unilateral involvement and for systemic therapy in bilateral cases with further therapies as adjuncts. 1 Monitoring for response is challenging, and formal guidelines for treatment and for defining residual disease after treatment remain to be established.…”

“… 1 Vitreoretinal lymphoma was known in the 1980s–1990s as reticulum cell sarcoma, 1 and it is often classified as either primary or secondary. 1 , 2 Vitreoretinal lymphoma is rare, as there are between 30 and 380 cases yearly in the United States, of which 95% are CD20 + , DLBCL. 1 , 3 Whereas choroidal and iridial lymphomas are usually low-grade with favorable prognoses, vitreoretinal lymphoma is typically high-grade with a worse prognosis.…”

“… 1 , 3 Whereas choroidal and iridial lymphomas are usually low-grade with favorable prognoses, vitreoretinal lymphoma is typically high-grade with a worse prognosis. 2 , 4 , 5 The brain is ultimately involved in 80% of cases, with one-third of patients showing CNS involvement within five years of diagnosis. 2 , 6 …”

“… 2 , 4 , 5 The brain is ultimately involved in 80% of cases, with one-third of patients showing CNS involvement within five years of diagnosis. 2 , 6 …”

Occlusive retinal vasculitis: novel insights into causes, pathogenesis and treatment

“…Rarely, an intravascular variant of large B cell lymphoma, known as angiotropic lymphoma can cause an ORV like picture [64]. Primary vitreoretinal lymphoma has also been linked to occlusive retinal vasculitis and nonperfusion [65,66]. Amyloidosis can also cause retinal nonperfusion and retinal microangiopathy [67].…”