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Retinoblastoma: from bench to bedside

Published online by Cambridge University Press:  13 February 2004

Richard L. Hurwitz ,
Patricia Chévez-Barrios ,
Milton Boniuk ,
Murali Chintagumpala  and
Mary Y. Hurwitz
Richard L. Hurwitz
Affiliation:
Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Patricia Chévez-Barrios
Affiliation:
Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Milton Boniuk
Affiliation:
Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Murali Chintagumpala
Affiliation:
Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Mary Y. Hurwitz
Affiliation:
Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
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Abstract

Retinoblastoma (Rb) is the most common primary ocular malignancy of children and is caused by a mutation in the gene RB1. Approximately 40% of cases are associated with one or more constitutional mutations, and are therefore heritable, whereas the other 60% are sporadic. Rb is exclusively found in young children. In some cases, Rb tumours metastasise to extraocular organs including bone, lung and brain. Although there is no effective treatment for metastatic disease, non-metastatic cases can be cured by removal of the eye (enucleation). Newer treatment strategies emphasise salvaging the affected eye whenever possible. Animal models of Rb have been developed with xenograft and transgenic techniques. Each model has both strengths and weaknesses for exploring the mechanisms of disease development and progression and the efficacy of new treatment strategies.

Keywords

retinoblastomagene therapyeyecancer
Type
Review Article
Information
Expert Reviews in Molecular Medicine , Volume 5 , Issue 1 , 7 January 2003 , pp. 1 - 22
Copyright
© Cambridge University Press 2003

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