Retinoblastoma Survival in Iran: 10 Years Experience of a Referral Center

Purpose: To describe the survival characteristics and prognostic factors of patients with retinoblastoma in a referral center in IranMethods: From medical records, we retrospectively analyzed the data of 139 consecutive children diagnosed in our hospital between 1991 and 2001 as having retinoblastoma. Information on gender, laterality, age at diagnosis, presenting signs, tumor staging, treatment modality, survival rate, and family history were collected.Results: Eighty-eight (63.3%) of the cases were unilateral and 51 (36.7%) of the cases were bilateral. The mean age overall at the time of diagnosis was 26.9 months; in unilateral cases, 32.7 months; and in bilateral cases, 22.8 months. The most common presenting signs were leukocoria (72.7%), strabismus (12.2%), and proptosis (7.9%). Fifty-one percent of patients had extraocular extension. The 5-year cumulative survival rate was 69.62% and the 10-year overall survival rate was the same as the 5-year survival rate. Patients with an age at the time of diagnosis of ≤2 years, with stage I and with positive family history had significantly better survivals both at the 5 and 10 years analysis (P 0.002, P 0.002, P 0.041 respectively). None of the patients developed a secondary neoplasm.Conclusion: In this study the mortality rate of patients with retinoblastoma is higher than the reports from developed countries. Availability and quality of registration data on retinoblastoma need to be improved for effective quantitative evaluation of incidence and survival