BACKGROUND: The timely and correct diagnosis of intraocular non-Hodgkin's lymphoma represents a huge challenge to clinicians. Two thirds of intraocular lymphomas are a manifestation of a primary CNS lymphoma (PCNSL) arising outside the lymphatic system and are localized in the brain, the meninges or the spinal chord. Ten to twenty percent commence as vitreous or retinal infiltrates mimicking uveitis. Ninety five percent of PCNSL are B cell lymphomas. PATIENTS: Three exemplary cases from a group of ten patients treated between 1998 and 2002 are presented. A table provides a summary of the relevant details of all ten patients. RESULTS: The mean age at presentation was 63.5 years with a female to male ratio of 6 to 4. Nine patients were diagnosed as having intermediate or posterior uveitis, in one patient choroidal metastases were suspected. Six patients had a concomitant CNS lesion while four patients showed isolated intraocular lymphoma only. The presence of a highly malignant B cell lymphoma was proven by vitreous biopsy in nine cases and by stereotactic biopsy of a CNS lesion in one patient. All patients were treated by intravenous chemotherapy, however, no binding recommendations with regard to treatment exist to date. CONCLUSIONS: We give an overview of all current treatment regimens and their pitfalls. At present it is recommended that all patients with proven PCNSL be entered in a multicenter randomized study under the auspices of the Department of Internal Medicine III of the Benjamin-Franklin-University-Hospital, Berlin and the Department of Neurology of the University Hospital of Tuebingen.
BACKGROUND: The timely and correct diagnosis of intraocular non-Hodgkin's lymphoma represents a huge challenge to clinicians. Two thirds of intraocular lymphomas are a manifestation of a primary CNS lymphoma (PCNSL) arising outside the lymphatic system and are localized in the brain, the meninges or the spinal chord. Ten to twenty percent commence as vitreous or retinal infiltrates mimicking uveitis. Ninety five percent of PCNSL are B cell lymphomas. PATIENTS: Three exemplary cases from a group of ten patients treated between 1998 and 2002 are presented. A table provides a summary of the relevant details of all ten patients. RESULTS: The mean age at presentation was 63.5 years with a female to male ratio of 6 to 4. Nine patients were diagnosed as having intermediate or posterior uveitis, in one patientchoroidal metastases were suspected. Six patients had a concomitant CNS lesion while four patients showed isolated intraocular lymphoma only. The presence of a highly malignant B cell lymphoma was proven by vitreous biopsy in nine cases and by stereotactic biopsy of a CNS lesion in one patient. All patients were treated by intravenous chemotherapy, however, no binding recommendations with regard to treatment exist to date. CONCLUSIONS: We give an overview of all current treatment regimens and their pitfalls. At present it is recommended that all patients with proven PCNSL be entered in a multicenter randomized study under the auspices of the Department of Internal Medicine III of the Benjamin-Franklin-University-Hospital, Berlin and the Department of Neurology of the University Hospital of Tuebingen.