Treatment of Synchronous Trilateral Retinoblastoma

Trilateral retinoblastoma is usually associated with a pineal lesion or, less commonly, a suprasellar lesion.[4-6] In patients with the heritable form of retinoblastoma, CNS disease is less likely the result of metastatic or regional spread than of a primary intracranial focus, such as a pineal tumor. The prognosis for patients with trilateral retinoblastoma is very poor. Most patients die of disseminated neuraxis disease in less than 9 months.[7,8] However, with increased surveillance and aggressive therapy, there has been improvement in survival, from 6% (patients treated before 1995) to 44% (patients treated after 1996).[9]

Treatment options for synchronous trilateral retinoblastoma include the following:

While pineoblastomas occurring in older patients are sensitive to radiation therapy, current strategies are directed towards avoiding radiation by using intensive chemotherapy followed by consolidation with myeloablative chemotherapy and autologous hematopoietic progenitor cell rescue. This approach is similar to those being used in the treatment of brain tumors in infants.[10]

For more information about trilateral retinoblastoma, including screening with neuroimaging, see the Trilateral retinoblastoma section.