Primary CNS Lymphoma with Intraocular Involvement

This is a rare entity that must be diagnosed and treated early.

Intraocular involvement in primary CNS lymphoma (PCNSL) is rare, and thus its natural history is unclear. These authors retrospectively reviewed data on 221 immunocompetent patients seen at 16 centers in 7 countries over a 28-year period.

The most common presenting symptoms were ocular (decreased or blurred vision or floaters), occurring in 62% of patients. The diagnosis is seldom made by history alone: Diagnosis required brain biopsy in 147 patients, vitrectomy in 65, and CSF cytologic examination in 11. Of 176 patients with treatment information available, 102 received dedicated ocular therapy (radiotherapy or intravitreal methotrexate) in addition to therapy targeted at the brain involvement; 72 received only the latter. Ocular therapy si…

Reviewing Authors

Peter Y. Chang, MD, C. Stephen Foster, MD

Fred H. Hochberg, MD