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Abstract Retinoblastoma (RB) is a malignant tumor of the developing retina that occurs in children, usually before age 5 years, and may be unilateral or bilateral. RB is the most common primary intraocular malignancy of infancy and childhood with an incidence of 1 in every 20,000 live births. Several classifications of RB have been developed to assist in prediction of globe salvage with preservation of useful vision where possible. There are two main classifications for intraocular retinoblastoma currently in use: 1. Reese-Ellsworth classification. ( R-E ) 2. International Intraocular RB Classification (IIRC) Children who are diagnosed as RB or suspected to have RB should undergo special investigations which will confirm the diagnosis and help in the treatment plan. Comprehensive management of RB requires a multidisciplinary approach. The combined efforts of the ophthalmologist, pediatric oncologist, radiation oncologist and ocular pathologist are aimed at saving the child’s life, the affected eye and if possible, vision. The currently available treatment methods for RB include: 1. Focal therapy 2. Systemic Chemotherapy: • Intravenous chemotherapy • Intra-arterial chemotherapy (IAC) 3. External Beam Radiotherapy (EBR) 4. Enucleation Group C cases are considered borderline, as some of them have good visual prognosis and respond properly to the chemotherapy, while others show progression, which may endanger child’s life itself. RB prognosis was very poor in the 19th century. Now survival has dramatically increased from around 5 percent to over 95 percent in the developed countries, making RB one of the success stories in childhood cancer. Now it has become apparent that chemoreduction is most successful for group C RB. Chemoreduction and focal conservative treatment for retinoblastoma provide impressive control for eyes with group C RB and BCVA up to 20/40. IAC is a recent very effective modality for the treatment of group C RB tumors achieving globe salvage with few systemic side effects. IAC appears favorable for group C RB. |