Retinoblastoma: Diagnosis, Classification and Management

Abstract

Retinoblastoma is the most common primary intraocular malignancy of childhood. Although a potentially fatal disease, its treatment has improved significantly in recent times. While saving life remains the primary goal, there is an increasing trend worldwide towards globe salvage and preservation of vision. This has been made possible since the introduction of intravenous chemotherapy and focal therapy. Targeted routes of drug delivery to the affected eye such as selective intra-arterial and intra-vitreal chemotherapy have been successfully employed. Plaque brachytherapy and external beam radiotherapy have an established role in selected cases. Enucleation is one of the most commonly performed procedures in advanced cases and the importance of post-operative adjuvant therapy in achieving better cure rates in children with high risk histopathological features has been recognized. Orbital disease remains a major challenge for the developing world. Nevertheless, multimodal therapy has improved survival in children with extra-ocular disease. This chapter provides an insight into the diagnosis of retinoblastoma, the various classification systems in use for staging the disease, as well as a brief description of the different treatment options that are available, including recent advances. Knowledge of these aspects is important for the practising ophthalmologist to acquire a clear understanding of the disease, its clinical presentation and management.