1. Mak KS, 1 Miller RC, 2 Krishnan S, 3 Laperriere N, 4 Micke O, 5 Rutten I, 6 Kadish SP, 7 Ozsahin M, 8 and Mirimanoff RO 8 1 Harvard Medical School, Boston, MA, 2 Mayo Clinic, Rochester, MN, 3 University of Texas M. D. Anderson Cancer Center, Houston, TX, 4 Princess Margaret Hospital, Toronto, ON, Canada, 5 Franziskus Hospital, Bielefeld, Germany, 6 Centre Hospitalier Universitaire de Liège, Liège, Belgium, 7 University of Massachusetts Medical School, Worcester, MA, 8 University Hospital Center and University of Lausanne, Switzerland OUTCOMES AND PROGNOSTIC FACTORS IN PRIMARY INTRAOCULAR LYMPHOMA: A MULTICENTER RARE CANCER NETWORK STUDY OBJECTIVE Primary intraocular lymphoma (PIOL) is a rare, malignant non-Hodgkin’s lymphoma, representing a subset of primary central nervous system lymphoma (PCNSL). Its natural history and outcome are thought to be worse than that of intraorbital lymphoma (Martinet et al., IJROBP, 2003), based on very small series or case reports. The goal of this study was to assess the clinical profile, treatment outcome, and prognostic factors in patients with Ann Arbor Stage I-E PIOL treated with radiation therapy and/or chemotherapy. MATERIALS & METHODS CONCLUSIONS PIOL has distinct clinical features and outcome, and its overall prognosis is fair. After therapy, local control was good but the rate of CNS recurrence was high, occurring in half of cases. Vitreous involvement carries a worse prognosis. CNS prophylaxis as well as more aggressive and innovative therapies need to be considered in this rare lymphoma entity. Male/Female cases Median age Median follow-up Median time from onset of symptoms to diagnosis 10/10 62 years 46.5 months 10 months (n = 20) (range: 48-82) (range: 8-151) (range: 1-26) Common symptoms Decreased visual acuity Blurred vision Floaters Masquerading Diagnoses Uveitis Vitritis Treatment Radiation therapy Radiation and chemotherapy Chemotherapy alone Radiation therapy Median dose Tumor characteristics Bilateral disease Location in vitreous Location in choroid, iris, ciliary body, or retina B cell origin reported n 14 4 2 n 9 12 8 16 n 20 15 6 n 12 7 37 Gy % 100 75 30 % 60 35 % 70 20 10 (range: 16-46) % 45 60 40 80 RESULTS In a median follow-up period of 46.5 months: Cases alive without disease45%n=9 Cases alive with disease25%n=5 Cases dead with disease25%n=5 Cases dead without disease5%n=1 Late Toxicities: Cataracts 20%n=4 Retinopathy 10% n=2 Dry eye syndrome 5%n=1 Survival Rates (Figure 1): Median overall survival 79 months 5-year overall survival 55% 5-year disease free survival39% 5-year local control rate72% Recurrence Rates: Local recurrence20%n=4 CNS recurrence (Figure 2)50%n=10 Systemic recurrence5%n=1 5-year probability of CNS relapse51% Case report of PIOL with CNS recurrence A 60-year old man was diagnosed with bilateral PIOL, confirmed by cytology (Figure 3A) and PCR as B cell in origin. Work-up was negative for any other lymphoma site. RT (36 Gy in 18 fractions) was delivered to both eyes, with complete remission. One year later, the patient developed a left hemiparesis. MRI disclosed a right periventricular mass (3B), consistent with lymphoma spread to the brain. High- dose methotrexate resulted in partial remission. Consolidation RT was then administered with a total dose of 50.4 Gy (3C, 3D). Figure 3: Case report of PIOL with CNS recurrence 3A: Cytology from sampling of vitreous fluid, revealing abnormal lymphoid cells with enlarged nuclei 3B: Brain MRI demonstrating R periventricular mass 3C, 3D: 3-D RT planning of brain lesion Figure 1: Local control rate, overall survival, and disease-free survival for the series of twenty patients Figure 2: Cumulative rate of CNS relapse in the series of twenty patients www.rarecancer.net