Osteosarcoma

OSTEOSARCOMA

Edited by Manish Agarwal

OSTEOSARCOMA Edited by Manish Agarwal

Osteosarcoma Edited by Manish Agarwal

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 Ivana Zec Technical Editor Teodora Smiljanic Cover Designer InTech Design Team First published April, 2012 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from [email protected]

Osteosarcoma, Edited by Manish Agarwal p. cm. ISBN 978-953-51-0506-0

Contents Preface VII Part 1 Chapter 1

Chapter 2

Part 2

Osteosarcoma Workup

1

Histopathology and Molecular Pathology of Bone and Extraskeletal Osteosarcomas Helen Trihia and Christos Valavanis

3

Imaging Osteosarcoma 41 Ali Nawaz Khan, Durr-e-Sabih, Klaus L. Irion, Hamdan AL-Jahdali and Koteyar Shyam Sunder Radha Krishna Osteosarcoma Treatment

83

Chapter 3

Misdiagnosis and Mistreatment for Osteosarcoma: Analysis of Cause and Its Strategy 85 Yao Yang and Lin Feng

Chapter 4

Chemotherapy in Osteosarcoma 93 Kapadia Asha, Almel Sachin and Shaikh Muzammil

Chapter 5

Limb Salvage for Osteosarcoma: Current Status with a Review of Literature 109 Manish G. Agarwal and Prakash Nayak

Part 3 Chapter 6

Chapter 7

Osteosarcoma Research

137

Bone Formation Deregulations Are the Oncogenesis Keys in Osteosarcomas Natacha Entz-Werlé

139

The Retinoblastoma Protein in Osteosarcomagenesis Elizabeth Kong and Philip W. Hinds

159

Preface Osteosarcoma, is the most common malignant primary bone tumor. Though high grade and potentially lethal, it is highly curable. Though a rare cancer, it predominantly affects children and young adults and being highly curable, the treatment strategy has permanent impact on the life of the patient. A plateau has been reached in its outcome but a lot of research is now being aimed at molecular level to find potential targets for treatment. The dissemination of knowledge coupled with availability of affordable chemotherapeutic drugs and prosthesis have improved the outcomes even in the developing world. The necessity of providing low cost treatment has resulted in innovation in strategy and surgical approach. Osteosarcoma is to orthopaedic oncology what tuberculosis is to infection. Both have varied spectrum of presentation and can mimic a host of conditions. This book is not meant to be a treatise on osteosarcoma. As the title suggests, it is a fresh look at this disease with an aim to update the existing knowledge on etiology, pathology and workup and to provide a look at the chemotherapeutic and surgical strategy. Importance has also been given to the new research which could potentially change the systemic treatment. What is also different about this book is that it is online, open access and has been done in a short turnaround time keeping the information very current at time of publication. The authors have published in this field before and many have experience of seeing hundreds of osteosarcomas. This book should be useful to clinicians, researchers as well as students with an interest in the field of osteosarcoma.

Manish Agarwal Hinduja Hospital, Mumbai, India

Part 1 Osteosarcoma Workup

1 Histopathology and Molecular Pathology of Bone and Extraskeletal Osteosarcomas Helen Trihia and Christos Valavanis

“Metaxa” Cancer Hospital, Department of Pathology and Molecular Pathology Unit Greece 1. Introduction Osteosarcoma has been recognized for almost two centuries and is the most common primary, non-haemopoietic malignant tumour of the skeletal system. It is thought to arise from primitive mesenchymal bone-forming cells and its histologic hallmark is the production of osteoid. Other cell populations may also be present, as these types of cells may also arise from pluripotential mesenchymal cells, but any area of bone or osteoid synthesized by malignant cells in the lesion establishes the diagnosis of osteosarcoma (Acchiappati et al, 1965). It accounts for approximately 20% of all primary malignant bone tumours. In the United States, the incidence of osteosarcoma is 400 cases per year (4.8 per million population