Abstract

Primary intraocular lymphoma is an uncommon clinical entity with poor visual and systemic prognosis. Optimal management of intraocular lymphoma remains uncertain. Three patients with intraocular lymphoma, two of whom had documented CNS involvement, were treated based on a modification of the Sloan-Kettering Cancer Center protocol for primary CNS lymphoma. All patients underwent diagnostic pars plana vitrectomy and histopathologic confirmation of primary intraocular large B-cell lymphoma. Treatment involved systemic chemotherapy with methotrexate and high-dose ARA-C, radiation therapy of the brain and orbits, and intrathecal methotrexate delivered via an Ommaya reservoir. Resolution of the ocular lymphoma was seen in all three patients, and resolution of the intracranial disease also was seen in the two patients with CNS involvement. All patients have remained disease free, with lymphoma in remission for at least 24 months after completion of treatment. The Sloan-Kettering protocol for the treatment of primary CNS lymphoma also appears to be effective in some cases of primary intraocular large cell lymphoma. Furthermore, the Ommaya reservoir works well for intrathecal delivery of methotrexate in patients with CNS or leptomeningeal spread.

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