[Retinal lymphoma--simulating uveitis]

A rare form of malignant large-cell non-Hodgkin's lymphoma or reticulum cell sarcoma is confined to the eye and central nervous system (CNS). We report the case of a 62-year-old man, who presented with recalcitrant progressive uveitis in his right eye. Although lymphoma was suspected, the patient refused to undergo vitrectomy. Over one year he developed large blister-like solid scattered lesions of the retinal pigment epithelium (RPE) in the same eye. Repetitive neuroimaging detected no CNS involvement. After developing optic disc-swelling and discrete RPE-changes in the left eye also, the patient agreed to enucleation of his right eye. Histopathology and Immuno-histochemistry revealed malignant intraocular large cell-lymphoma invading all eye-tissues. Liquor cytology showed lymphomatous cells. Suffering from high grade malignant lymphoma with CNS ivolvement, the patient had to undergo radiation therapy of the orbits and neuroaxsis as well as intravenous and intrathecal chemotherapy. Papilledema of the left eye resolved quickly and completely, RPE-infiltrations disappeared and led to discrete punched out lesions with no scarring, the lens remained transparent over the whole observation period of 25 months. the patient has been surviving for more than 52 months after first suspicion of malignant lymphoma, he has retained full vision in his left eye until now.