Purpose. To examine the histopathogenesis, treatment modalities and survival in 71 cases of trilateral retinoblastoma (TRb) reported in the world literature. Methods. We reviewed all cases of TRb reported from 1977-1996. Results Histopathologic findings demonstrate primitive neuroectodermal tumors in 55% of cases. Varying degrees of photoreceptor differentiation were seen in 45% of cases. Autopsy, histopathologic and radiologie examination do not reveal a definitive origin, although "pinealoblastoma" was often the diagnosis reported. Histopathologic evidence of subependymal\ependymal craniospinal seeding was seen in 65% of cases. TRb is uniformly fatal with an average survival of 11.2 months. Therapy included: 1) radiation, 2) systemic chemotherapy, and 3) surgical resection/ craniotomy in combination with radiation and/or chemotherapy with survival of 5.4, 25.8 (4 children alive at time of report), and 16.8 months (2 children alive), respectively. Patients receiving platinum-based systemic chemotherapy in combination with intrathecal chemotherapy and neural axis radiation demonstrated an average survival of 15.5 months. Conclusion. TRb more likely arises from a germinal layer of predisposed primitive subependymal neuroblasts that are not necessarily destined for pineal or photoreceptor differentiation. Recent success with platinum-based ocular chemoreduction together with the above findings, suggests that routine CNS imaging and aggressive platinum-based chemotherapy, intrathecal chemotherapy, radiation and surgery should be considered.
|Original language||English (US)|
|Journal||Investigative Ophthalmology and Visual Science|
|State||Published - 1997|
ASJC Scopus subject areas
- Sensory Systems
- Cellular and Molecular Neuroscience