Retinoblastoma: genetic considerations and report of a new animal model

Abstract

Retinoblastoma is the most common primary, intraocular neoplasm of childhood. Histologically, retinoblastoma resembles, in many respects, other pediatric malignancies such as medulloblastoma and neuroblastoma. These tumors are composed of small, basophilic cells with scanty cytoplasm and often form non-specific Homer Wright rosettes. Retinoblastomas frequently possess, in addition, the distinctive Flexner-Wintersteiner rosettes, a form of photoreceptor differentiation. Retinoblastoma was uniformly fatal until the second half of the nineteenth century [1]. Improvement in prognosis occured as a result of earlier diagnoses made possible by the ophthalmoscope and the adoption of enucleation with an adequate segment of the optic nerve as the primary treatment for retinoblastoma [1]. As children with retinoblastoma survived to adulthood, the role of heredity in retinoblastoma was appreciated.