Abstract
BackgroundPrimary vitreoretinal lymphoma (PVRL) is a rare ocular condition and its diagnosis remains a challenge. The clinical presentation is variable and it can masquerade as chronic intermediate or posterior uveitis. We report an unusual case of primary central nervous system lymphoma (PCNSL) presenting as migrating retinal lesions with unique shapes. The diagnostic challenges are described and the clinical features of intraocular lymphoma are reviewed.Case presentationA 53 year-old gentleman presented with unilateral visual disturbance and a wide area of retinal whitening with sharp borders temporal to the macula, corresponding to hyper-reflective subretinal changes on optical coherence tomography (OCT). The lesion resolved spontaneously after 6 weeks but was replaced by multiple punctate sub-retinal and sub-retinal pigment epithelial lesions. The second eye was involved 4 months later and there were new areas of hyper-reflective changes in both eyes, which migrated nasally within a week, with no evidence of scarring in the previous sites. The diagnosis of diffuse B-cell lymphoma was made on brain biopsy when the patient subsequently presented with acute confusion and magnetic resonance imaging brain scan showed a frontal lesion. Following systemic chemotherapy, the sub-retinal changes resolved with complete restoration of retinal architecture and improvement in visual acuity.ConclusionWe report an unusual case of PVRL presenting as migrating retinal lesions with unique shapes. PVRL is a great imitator and a high index of clinical suspicion is required in unexplained ocular lesions to prevent a delay in diagnosis.
Highlights
Primary vitreoretinal lymphoma (PVRL) is a rare ocular condition and its diagnosis remains a challenge
We report an unusual case of PVRL presenting as migrating retinal lesions with unique shapes
Primary central nervous system lymphoma (PCNSL) is a rare malignancy that is confined to the brain, spinal cord, eyes, and leptomeninges
Summary
Primary central nervous system lymphoma (PCNSL) is a rare malignancy that is confined to the brain, spinal cord, eyes, and leptomeninges. Significant evolution of the right lesion morphology was observed: the temporal margin appeared more defined with re-emergence of a bright linear edge; the lesion had increased in size; new sub-retinal deposits were identified (Fig. 1c) and the lesion appeared to have migrated supero-nasally across the peri-papillary area, maintaining the outline shape temporally of the earlier lesion. Within the previously involved area, the sub-retinal lesion clinically appeared to have resolved and OCT demonstrated resolution of the hyper-reflective change with the RPE intact. Clinical examination and repeat imaging demonstrated resolution of the large retinal lesions and reduced number of sub-RPE lesions in the both eyes with complete restoration of the outer retinal architecture on OCT (Fig. 3a to d)
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