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Background: Retinoblastoma is the most common embryonic tumor of retina of children. This tumor include 3-4% of all children malignancies during the age range 0-14 years. In this study, we reported the incidence, therapeutic protocols, and treatment outcome of children with RB in South west Iran. Methods: We retrospectively investigated the medical reports clinical and pathological features of RB cases at referral Shafa Hospital of Ahvaz city located in Southwest Iran, between 1994 and 2014. Results: Our results showed leukocoria, white discoloration in the pupil, as the most common primary symptom in retinoblastoma patients. Forty-two percent of our patients had bilateral neoplasms. Based on histological investigations, stage IV was most common stage in our patients (79.1%). Conclusion: In conclusion, our study showed that leukocoria, stage IV and Choroid involvement as most common symptom, stage and organ involvement, respectively and inherited cases is rare in our population. The results of our study can be as a base for further researches in future. Keywords: Retinoblastoma, clinical manifestation, incidence, heritable, staging, treatment modality

Keywords: Retinoblastoma, clinical manifestation, incidence, heritable, staging, treatment modality

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