Lymphoma of the Optic Apparatus in an Immunocompetent Patient: A Case Report and Review of the Literature

Background: Primary optic apparatus involvement by lymphoma is an exceedingly rare entity, with only 3 cases previously reported in the literature. Whether this represents a distinct pathology, metastatic disease from an unidentified systemic lymphoma, or the first manifestation of evolving primary central nervous system lymphoma is not currently understood.

Case description: We present a case of a young immunocompetent male with rapidly progressive visual loss who was found to have isolated lymphomatous involvement of the optic apparatus. We discuss the classification of the lesion, the clinical presentation, the diagnostic workup, and the visual and overall prognosis. Special consideration is given to the operative approach and selecting an appropriate site for biopsy based on the visual exam at the time of presentation.

Conclusions: Primary optic nerve lymphoma is a rare disease that requires a systemic workup and a multidisciplinary approach to treatment.