Retinoblastoma: An overview

Retinoblastoma is the most common intraocular malignancy in children, with a reported incidence ranging from 1 in 15,000 to 1 in 18,000 live births. It is second only to uveal melanoma in the frequency of occurrence of malignant intraocular tumors. Pawius described retinoblastoma as early as in 1597 referred to the tumor as fungus hematodes and suggested enucleation as the primary mode of management. The discovery of ophthalmoloscope in 1851 facilitated recognition of specific clinical features of retinoblastoma. Initially thought to be derived from the glial cells, it was called a glioma of the retina by Virchow (1864). Flexner (1891) and Wintersteiner (1897) believed it to be a neuroepithelioma because of the presence of rosettes. Later, there was a consensus that the tumor originated from the retinoblasts and the American Ophthalmological Society officially accepted the term retinoblastoma in 1926. Retinoblastoma was associated with near certain death just over a century ago. There has been a dramatic change in the overall management of retinoblastoma in the last decade. Specific genetic protocols have been able to make pre natal diagnosis of retinoblastoma. Early diagnosis and advancements in focal therapy have resulted in improved eye and vision salvage. This article explains the complexity of retinoblastoma, genetic association, clinical features, management and prognosis.