Simultaneous Intraocular and Orbital Non-Hodgkin Lymphoma in the Acquired Immune Deficiency Syndrome

Background: Non-Hodgkin lymphoma is more common in patients with the acquired immune deficiency syndrome (AIDS), and the incidence of non-Hodgkin lymphoma in the AIDS population has been increasing as the life expectancy of these patients has increased. Nevertheless, intraocular lymphoma, as part of primary central nervous system lymphoma and orbital non-Hodgkin lymphoma, has rarely been reported in patients with AIDS. Co-existent intraocular and orbital lymphoma is exceptionally unusual. The clinical and histopathologic findings are reported in a patient with AIDS who had simultaneous intraocular and orbital non-Hodgkin lymphoma. The clinical funduscopic findings initially were attributed to a viral retinitis. Methods: A left orbital biopsy followed by enucleation of the left eye was performed and studied by light microscopy. Immunophenotyping of the orbital biopsy also was performed. Findings: Simultaneous intraocular and orbital large cell malignant lymphoma was present with neoplastic spread directly along the scleral canal of the ciliary nerve. Massive necrosis involving the retina, retinal pigment epithelium, choroid, and optic nerve, as well as several solid retinal pigment epithelial detachments, were observed. Immunophenotyping of the orbital tumor disclosed positive staining for Leu 4 (T cells, 30%) and Leu 14 (B cells, 60%). Immunostaining for light and heavy chains was precluded by tumor necrosis. Conclusion: Non-Hodgkin lymphoma should be included in the differential diagnosis of acute retinitis, and proptosis in patients with AIDS.