Intra-Ocular Lymphoma with and without CNS Involvement: Diagnosis and Follow-up a Report of 15 Cases Studied by MR

Abstract

In order to assess the value of MRI in the diagnosis and follow-up of intra-ocular lymphoma, a rare form of primary non-Hodgkin's lymphoma of the central nervous system, we retrospectively reviewed fifteen patients. All patients had ophthalmic investigations and 13 underwent ocular sampling. MR examinations of the brain and globes were performed in all cases and five patients underwent stereotactic brain biopsy. Six patients were treated with chemotherapy and/or radiotherapy, and nine with high-dose chemotherapy, followed by autologous bone marrow transplantation in five cases. MR follow-up was available in all cases. All 15 patients had chronic uveitis which preceded the diagnosis and abnormal funduscopic findings. Three had a mild or severe neurologic deficit. Initial MRI showed brain lymphoma lesions in six cases and a choroido-retinal tumour in one. MR brain lesions were multiple in four cases. They appeared as contrast-enhanced infiltrating areas (n=11) or expansive masses (n=3); two lesions appeared as infiltrating high-signal T2 areas but were unenhanced on T1 with GdDTPA. The diagnosis was based on vitrectomy in 11 cases and on stereotactic brain biopsy in four. Of the twelve lumbar punctures which were performed one was positive. Contrast enhancement disappeared during treatment in all cases, but isolated signal abnormalities persisted. The long-term outcome of such lesions in patients with an intact blood-brain barrier is not yet known. Ocular relapses occurred in 14 patients and CNS recurrences in four. Three patients died from CNS failure (n=1) or relapse (n=2), five are alive in partial remission, five are in complete remission and two died in remission from other causes. Follow-up ranges from 12 to 78 months (median 36 months). MRI usually failed to detect intra-ocular lesions but identified clinically occult brain lesions and served to guide stereotactic brain biopsy when other samples were negative. MRI is the most sensitive follow-up method during treatment, even when the blood-brain barrier is intact.