A healthy 41-year-old male presented with a 2-year history of increasingly blurry vision in the right eye. His review of systems was otherwise negative. He had a family history of skin cancer in his father and leukemia in his cousin. Visual acuity was hand motions in the right eye and 20/20 in the left eye. Pressure was 16 OU. Slit-lamp biomicroscopy showed trace nuclear sclerotic cataracts in both eyes with no other abnormalities. Dilated examination of the right eye revealed a retinal detachment and an amelanotic subretinal mass extending from the posterior pole and tapering into the periphery (fig. 1).
Color fundus photography revealed an exudative retinal detachment and an elevated whitish subretinal/choroidal mass lesion in the posterior pole. Fluorescein angiography revealed early hypofluorescence with minimal late hyperfluorescence. An MRI of the brain and orbit showed a 5-mm right choroidal mass in the posterior globe with no other intracranial findings, and OCT also revealed choroidal thickening. All of these findings were highly suggestive of uveal lymphoma and help to differentiate this lesion from primary vitreoretinal lymphoma, which typically shows hyperreflective nodules at the RPE level and sub-RPE space on OCT, and clusters of round, hyper- or hypofluorescent lesions on fluorescein angiography. The remainder of the workup was negative, including CT of the chest/abdomen/pelvis, CMP, LDH, CBC with differential, ACE, RPR, and quantiferon TB-gold.
Transcleral fine needle aspiration biopsy of the choroidal lesion was performed. Immunohistochemical staining of the cell block revealed a population of predominantly CD20-positive B lymphocytes with comparatively fewer CD3- and CD5-positive T cells. No co-expression of CD5 and BCL-1 was appreciated. IgH gene rearrangement for IgH V-D-J revealed a monoclonal population of B lymphocytes, consistent with lymphoma (fig. 2).
The patient was treated with external beam radiation therapy (EBRT). Imaging obtained 4.5 months after ERBT showed excellent regression of the uveal mass, significantly reduced thickness of the choroidal lesion, resolution of the retinal detachment, and no signs of subretinal fluid. OCT demonstrated residual subretinal scarring (fig. 1). The patient's visual acuity remained hand motions.