Intraocular lymphomas. Natural history based on a clinicopathologic study of eight cases and review of the literature.

Eight patients with intraocular lymphoma were studied; all were middle-aged or elderly, usually presenting with a unilateral visual deficit. All patients also had an extraocular lymphoma with either systemic (2) or isolated central nervous system (CNS) involvement (6). When ocular symptoms preceded CNS symptoms, they presented from 11 months to 10 years earlier. Intraocular lymphoma tended to affect the eye ipsilateral to the CNS tumor either exclusively or initially, and to the greatest degree histologically. Histologic examination of intervening optic pathways revealed extension of intraocular tumor across the optic nerve into orbital leptomeninges in 3 cases. Corresponding CNS tumors showed prominent leptomeningeal involvement. All intraocular and extraocular tumors showed diffuse growth patterns with histiocytic (large cell), poorly differentiated lymphocytic or mixed lymphocytic-histiocytic subtypes. CNS tumors were detected only after onset of neurologic symptoms, and were almost always fatal, although CNS irradiation prolonged survival for years in some cases. Our data suggest that the peculiar link between intraocular and CNS lymphomas may reflect both multicentric and metastatic disease processes. The prompt recognition of intraocular lymphomas as a harbinger of extraocular tumor may prove valuable in earlier recognition and treatment of the disease.