Large B-cell lymphoma of the uvea: Histopathologic variants and clinicopathologic correlation

Twenty-eight patients were identified with large B-cell lymphoma of the uvea. Uveal involvement was iris (1 case), ciliary body (1 case), or choroidal in 14 cases. Panuveal involvement was observed in 12 cases. The clinical presentation could be categorized into uveitis (8), intraocular mass (9), neovascular glaucoma (4), and vascular disorders (4). The majority (21 cases, 77%) were diagnosed at autopsy (11) or after enucleation (10). Only 7 were diagnosed with conservative techniques. Histopathologically, 3 distinct subgroups of large B-cell lymphoma could be identified: 15 were characterized as diffuse large B-cell lymphoma, 11 as intravascular large B-cell lymphoma, and 2 as plasmablastic lymphoma. All cases had a poor prognosis, with a median survival of 14 months. Most cases (19, 67%) represented secondary uveal involvement with widespread systemic lymphoma at ophthalmic presentation. Six cases were treated with radiotherapy, most of these diagnosed before the 1990s (4). Subsequent cases (9) received systemic or local chemotherapy and adjunct radiotherapy, depending on the organs affected. Two cases were treated only with enucleation, and systemic treatment was not specified in 13 cases. Large B-cell lymphoma can rarely involve the uvea. The presenting features are nonspecific, often leading to enucleation. Effective therapy is not known. In all 3 variants, the aggressive nature and widespread involvement at ophthalmic presentation is associated with short survival.