• The management of retinoblastoma has become more complex in the era of primary systemic chemotherapy.
• The management of retinoblastoma requires a multidisciplinary team composed of an ophthalmologist, pediatric oncologist,
and radiation oncologist.
• Enucleation remains the standard of care for patients with massive unilateral disease in which the affected eye has limited
visual potential and the other eye is normal.
• Any eye with limited visual potential in which retinoblastoma cannot be excluded should be enucleated.
• Retinoblastoma is a systemic disease; germ-line mutations of the RB1 gene lead to 30% mortality from second cancers.