Abstract
In the management of retinoblastoma, it is important to remember that there is an entire patient to treat – not just an intraocular tumor. Children with heritable retinoblastoma who have a germ-line mutation in RB1 have an increased risk of developing a second malignancy. All familial cases, all bilateral cases, and approximately 15 % of unilateral cases fall into this category. The most common secondary malignancies are sarcomas, melanoma, and brain tumors. The association of retinoblastoma and an intracranial neuroblastic malignancy, known as “trilateral retinoblastoma,” most often involves the pineal gland (“pineoblastoma/pinealoblastoma”) but may also involve parasellar and suprasellar regions (10.1007/978-3-662-43451-2_20) [1–3]. The increased risk of developing second, non-ocular malignancies for survivors of heritable retinoblastoma is estimated to be 20 times higher than the general population [4].