Tumor Models of Retinoblastoma: In Vivo, Ex Vivo, and In Vitro Models

In the initial simplistic understanding, childhood retinal cancer retinoblastoma is due to the defect in the retinoblastoma gene. However, the deep insights reveal the complexity of the disease from unilateral single gene mutation to the bilateral two-hit hypothesis proposing two mutations in two alleles of the same gene and more recent studies revealing the role of multigene dysfunction in such cancers without any abnormality in the retinoblastoma gene. Again, retinoblastoma majorly occurs among humans while replicating the same disease among animals with the mutations in Rb gene shows more complexity to the evolution of the disease as the Rb gene mutations alone do not result in the diseased retina in the organism. Thus, it has become vital to develop various alternatives to study disease initiation, development, and treatment. Here we summarize the current understandings of the disease, in vitro, ex vivo, and animal models, and the standard treatment procedures as well as the development of novel therapeutics for such a complex disease.