Trilateral Retinoblastoma

Trilateral Retinoblastoma

Bilateral retinoblastomas often occur in the familial form of the disease, which is characterized genetically as a mutation in the Rb1 tumor suppressor gene. These patients are also at risk for developing a third intracranial tumor, usually in the pineal gland, the so-called third eye. Trilateral retinoblastoma involves tumor in both eyes and a metachronous PNET in the pineal gland. As in the retina, even a fleck of calcium in the pineal gland of a child younger than 6 years old should be considered suggestive of neoplasm.

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