Retinoblastoma : Top Ten Tenets for an Ophthalmologist
1.Common and Curable Retinoblastoma is the most common childhood ocular malignancy. Annually there are about 6000 new cases worldwide. It is most commonly diagnosed at 15-18 months of age although in developing countries, older children also present with the disease. It is potentially curable if detected early and with proper management. Currently, the survival rate is as high as 95% with 90% eye salvage and 85% vision salvage. 2 The root of the Family Tree Retinoblastoma can be associated with germline mutation or somatic mutations of the RB1 gene on the long arm of chromosome 13. Heritable retinoblastoma constitutes 30-40% of all retinoblastomas while the rest are non- heritable. 25% of the germline mutations are familial with autosomal dominant inheritance and the remaining occur de novo. In heritable retinoblastoma, patients are prone to other second primary neoplasia including pinealoblastoma, osteosarcoma and soft tissue sarcomas. Genetic testing for germline mutations are availa

