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Retinoblastoma: From the Two-Hit Hypothesis to Targeted Chemotherapy

¹ Department of Embryology, Carnegie Institution, Baltimore, Maryland; ² Department of Developmental Neurobiology, St. Jude Children's Research Hospital and ³ Department of Ophthalmology, University of Tennessee Health Sciences Center, Memphis, Tennessee

Requests for reprints: David MacPherson, Department of Embryology, Carnegie Institution, Baltimore, MD. E-mail: macpherson{at}ciwemb.edu and Michael A. Dyer, Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Mail Stop 323, 332 N. Lauderdale, Memphis, TN 38105-2794. Phone: 901-495-2257; Fax: 901-495-3143; E-mail: michael.dyer{at}stjude.org.

Studies on retinoblastoma have been at the heart of many of the landmark discoveries in cancer genetics over the past 35 years. However, these advances in the laboratory have had little effect on the treatment of children with retinoblastoma. One of the reasons for this has been the lack of preclinical models that recapitulated the genetic and histopathologic features of human retinoblastoma. In the past three years, a series of new animal models of retinoblastoma has been developed and characterized from several different laboratories using a variety of experimental approaches. It is encouraging that there is broad agreement about the consequences of inactivation of the Rb family in retinal development from these studies. More importantly, these new mouse models of retinoblastoma have contributed to clinical trials and novel therapeutic approaches for treating this debilitating childhood cancer. [Cancer Res 2007;67(16):7547–50]