RETINOBLASTOMA

AN UPDATE ON CLINICAL, GENETIC COUNSELING, EPIDEMIOLOGY AND MOLECULAR TUMOR

BIOLOGY

Edited by

Govindasamy Kumaramanickavel

RETINOBLASTOMA – AN UPDATE ON CLINICAL, GENETIC COUNSELING, EPIDEMIOLOGY AND MOLECULAR TUMOR BIOLOGY

Edited by Govindasamy Kumaramanickavel

Retinoblastoma – An Update on Clinical, Genetic Counseling, Epidemiology and Molecular Tumor Biology

Edited by Govindasamy Kumaramanickavel

Published by InTech

Janeza Trdine 9, 51000 Rijeka, Croatia

Copyright © 2012 InTech

All chapters are Open Access distributed under the Creative Commons Attribution 3.0 license, which allows users to download, copy and build upon published articles even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source.

As for readers, this license allows users to download, copy and build upon published chapters even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications.

Notice

Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published chapters. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book.

Publishing Process Manager Vana Persen

Technical Editor Teodora Smiljanic

Cover Designer InTech Design Team

First published March, 2012

Printed in Croatia

A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechopen.com

Retinoblastoma – An Update on Clinical, Genetic Counseling, Epidemiology and Molecular Tumor Biology, Edited by Govindasamy Kumaramanickavel

p. cm.

ISBN 978-953-51-0435-3

Contents

VI Contents

Chapter 8 Cytoskeletal Organization

and Rb Tumor Suppressor Gene 131

Yi-Jang Lee, Pei-Hsun Chiang

and Peter C. Keng

Chapter 9 Viral Oncogenes and the Retinoblastoma Family 155

M. Geletu and L. Raptis

Preface

Retinoblastoma is the first ever discovered tumor suppressor gene that opened a new avenue in the field of oncology leading to the identification of 35 tumor suppressor genes, till date in our genome. It is four decades since we know the two-hit hypotheses of Dr Alfred G Knudson and presently there is a huge amount of data available for us to completely comprehend the retinoblastoma gene and protein. However this reveals that there is more to learn and understand about its character and characteristics.

This book is an excellent compilation of both clinical and basic science information that meets the needs of a young clinician and a researcher at the same time. It also has abundant information on recent advances and cutting-edge knowledge in intracellular molecular cross-talking of retinoblastoma protein with various cellular viral-like proteins.

Looking into the details of this book, you will find that there is an excellent clinical description of the disease with adequate illustrations. The dreadful problem of second malignancies both ocular and non-ocular is dealt with elegantly. The ototoxic hearing loss in retinoblastoma patients provides greater insight into the disease, which would be a useful tool for practicing clinicians. For all levels of clinicians, whether entry, mid or senior, there is information on the genetic counseling and molecular diagnostics which are very useful. The epidemiology of retinoblastoma is a revelation for those both in clinics and research.

In understanding the molecular tumor biology of the disease, the role of RB protein in cell growth and tumor progression is extensively described. Interestingly a little known role of RB protein in the survival and differentiation of cerebellar neurons is discussed in great detail. The role of viral oncogenes and retinoblastoma family proteins is an exciting area that is teased out. The genetics of retinoblastoma is described quite elaborately as well.

Dr. Govindasamy Kumaramanickavel

Research Director, Narayana Nethralaya, Bangalore,

Advisor - Research, Academics and Management, Aditya Jyot Eye Hospital, Mumbai

India

Visiting Associate, Ophthalmic Genetics and Clinical Services Branch,

National Eye Institute, NIH

USA

Part 1

Clinical Sciences