ABSTRACT
Retinoblastoma is the most common primary ocular malignancy of childhood, occurring in about 1 in 18,000 live births in the United States. It is estimated that 200 new cases are diagnosed every year in the United States [1]. There is evidence that retinoblastoma may be more frequent in some developing nations, such as India [2] and in Latin America [3]. The survival of patients in developed nations diagnosed with retinoblastoma has improved over the last century, likely due to earlier diagnosis and improved treatment [1]. Most of these patients present with intraocular disease, while delayed diagnosis has been associated with an advanced stage of disease and a poorer prognosis [2,3]. The previous chapters in this book thoroughly discuss retinoblastoma, primarily intraocular. Therefore, this chapter focuses on extraocular disease.
