Retinoblastoma is an early-onset cancer of the retina with a rapid progression, and is mostly diagnosed in children. In its hereditary form, multiple eye tumors usually arise in both eyes, while the nonhereditary form usually causes fewer tumors in only one eye. Treatment may involve a combination of chemotherapy, radiotherapy, and other therapies and the majority of patients can be cured if given the right treatment. However, survivors of one form of retinoblastoma (and not the other form) have a markedly increased frequency of subsequent neoplasms that can lead to other cancers later in life, especially soft-tissue sarcomas. These patients should therefore be closely monitored throughout their lives. Which gene is affected by the primary driver mutation in this cancer as well as the later sarcomas? Which form of retinoblastoma do you think is associated with a higher risk of subsequent neoplasms?

Question

Quizplus · Accepted Answer

Due to a germ-line mutation in the Rb gene, people with hereditary retinoblastoma are at a higher risk of cancers in older age. Note that this is different from "relapse" of partially treated retinoblastomas, which can be accounted for by the survival of a few original cancer cells after the initial treatment.

Retinoblastoma Is an Early-Onset Cancer of the Retina with a Rapid