Factors That Influence Long Term Survival in Patients with Primary Intraocular Lymphoma

Abstract

Abstract: : Purpose: Primary intraocular lymphoma (PIOL) is a large B-cell, non-Hodgkin's primary central nervous system lymphoma (PCNSL) involving only the eyes. PIOL is a rare disease usually encountered in elderly population. PIOL has a poor visual outcome and the average survival after diagnosis is 3 years. Age, performance status and treatment modality have been reported as important prognostic factors in PCNSL. The purpose of this study was to determine the factors influencing long-term survival in patients with PIOL. Methods: A retrospective, non-comparative review of the clinical records of PIOL patients surviving > 6 months and followed at the NEI between 1991 and 2002. Results: Seven patients (5 female, 2 male) with a mean age of 51.4 years (range: 46-64 years) at the time of PIOL diagnosis were identified. 6/7 patients remain alive with a mean survival of 67 months (range: 30-128 mo) and the one death occurred 67 months after the PIOL diagnosis. PCNSL was present at the time of PIOL diagnosis in 5/7 and was diagnosed after 10 and 16 months in the remaining 2. Initial treatment in 4/7 patients included systemic chemotherapy with high dose methotrexate with adjunctive intrathecal chemotherapy in 2 and additional orbital radiotherapy following the systemic chemotherapy in 1. Initial therapy in the remaining 3 patients was whole brain and orbital radiation with one also receiving intrathecal chemotherapy. Recurrent PCNSL was documented in 3/4 patients treated initially with systemic chemotherapy and 2/3 patients initially treated with radiation. Five of the 7 patients underwent complete ophthalmic examination every 6-8 weeks. The average ocular recurrence rate in 4 of the seven patients was 2.2 per year over a mean follow-up time of 76.3 months. Cytokine analysis (IL-10, IL-6) for longitudinal follow-up on intraocular fluid specimens in 4 patients consistently correlated with the presence of PIOL clinically presence. Ocular recurrences in 6/7 patients were treated with intravitreal chemotherapy, usually methotrexate (dose range: 0.2 - 0.45 mg/0.1 ml). One patient underwent enucleation of one eye following intravitreal therapy. On last follow-up a visual acuity 20/60 or better was recorded in at least one eye in 5/7 patients. Conclusions: Based on this limited case series, younger patients (< 60 years)who are followed closely for their CNS or ocular recurrences, treated agressively and showed low IL-10/IL-6 ratios may have a longer survival. Early recognition and prompt treatment of ocular recurrences may preserve a functional vision in PIOL patients.

Keywords: tumors • uveitis-clinical/animal model