Choroidal extranodal marginal zone lymphoma diagnosed by full-thickness retinochoroidal biopsy: case report and review of the literature

Abstract

The case of an 89-year-old man who was referred for a painless decrease of vision in his right eye (RE) is reported. Fundus examination of the RE showed an elevated amelanotic lesion located in the posterior pole with an adjacent focal round pigmented lesion. There was also a more peripheral amelanotic lesion extending from 6 to 9 o’clock clockwise inferotemporally. Uveitis workup and imaging studies of brain and orbits were normal. A retinochoroidal biopsy was done and showed the presence of choroidal extranodal marginal zone lymphoma. The patient was treated with external beam radiotherapy. This report presents a review of the literature of all reported cases of choroidal extranodal marginal zone lymphoma.

Ultra-low-dose (boom-boom) radiotherapy for choroidal lymphoma in three consecutive cases

“…Regarding ultra-low-dose radiotherapy for uveal lymphoma, there has been only one prior case report of a 89-year-old male with primary choroidal lymphoma, confirmed on a full-thickness retinochoroidal biopsy, and treated with ultra-low-dose radiotherapy. 10 On 6-month follow-up, complete tumor regression was noted with visual acuity of counting fingers.…”

“…In the literature, there is only one prior description of ultra-low-dose radiotherapy for biopsy-proven choroidal lymphoma. 10 Herein, we describe our experience with ultra-low-dose radiotherapy in three consecutive patients with choroidal lymphoma.…”

Choroidal lymphoma diagnosed by polymerase chain reaction–based clonality assessment

“…1 The most used therapy in these cases is EBRT, and a large amount of reports support it to be the most effective therapy to manage CLs. 1–3,5,6 However, as our patient refused this possibility initially, we decided to use intravitreal rituximab (a chimeric monoclonal antibody that targets the CD20 protein found on the surface of B-cells). It has been reported a good option in systemic lymphomas and also in PVRLs.…”

“…1,2 EMZL generally involves the conjunctiva, lacrimal gland, and orbit, but intraocular choroidal involvement is rare and has rarely been reported. 3 Signs and symptoms of CLs can mimic other ocular diseases, such as choroidal amelanotic melanoma, uveal metastases, posterior scleritis, and posterior uveitis. 1,3 For the first therapeutic approach in those last diseases, corticosteroids are widely used.…”

“…3 Signs and symptoms of CLs can mimic other ocular diseases, such as choroidal amelanotic melanoma, uveal metastases, posterior scleritis, and posterior uveitis. 1,3 For the first therapeutic approach in those last diseases, corticosteroids are widely used. Since an initial improvement using steroids can be observed in primary CLs, the diagnosis is often delayed months or years.…”