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 orders@intechopen.com Osteosarcoma, Edited by Manish Agarwal p. cm. ISBN 978-953-51-0506-0 Contents Preface VII Part 1 Osteosarcoma Workup 1 Chapter 1 Histopathology and Molecular Pathology of Bone and Extraskeletal Osteosarcomas 3 Helen Trihia and Christos Valavanis Chapter 2 Imaging Osteosarcoma 41 Ali Nawaz Khan, Durr-e-Sabih, Klaus L. Irion, Hamdan AL-Jahdali and Koteyar Shyam Sunder Radha Krishna Part 2 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 Osteosarcoma Research 137 Chapter 6 Bone Formation Deregulations Are the Oncogenesis Keys in Osteosarcomas 139 Natacha Entz-Werlé Chapter 7 The Retinoblastoma Protein in Osteosarcomagenesis 159 Elizabeth Kong and Philip W. Hinds 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 [...]... first published by Trihia et al, Acta Cytologica, 2007;51:443-450) 9 10 Osteosarcoma 2.5 Secondary osteosarcomas Secondary osteosarcomas are bone forming sarcomas occurring in bones that are affected by preexisting abnormalities, the most common being Paget disease and radiation change 2.5.1 Paget osteosarcoma Incidence of sarcomatous change in Paget disease is estimated to 0.7-0.95%, with osteosarcomas... correct diagnosis 2.7 Periosteal osteosarcoma It accounts for less than 2% of all osteosarcomas, more common than high grade surface osteosarcoma, but about one third as common as parosteal osteosarcoma (Unni et al, 1976) Unlike parosteal osteosarcoma, which extends from the cortex like a bony knob, periosteal osteosarcoma tightly encases the bone, like a glove Unlike other osteosarcomas, it tends to involve... et al., 2001) 22 Osteosarcoma 5.4.3 Small cell osteosarcoma No specific cytogenetic abnormalities have been detected in small cell osteosarcoma Small cell osteosarcoma can be distinguished from Ewing sarcoma by the presence of neoplastic bone and the absence of the t(11:22) translocation or one of its variants 5.4.4 Low grade central osteosarcoma Low grade central (intramedullary) osteosarcoma show... Telangiectatic osteosarcoma When first recognized, telangiectatic osteosarcoma was considered a distinct clinical and pathologic entity (Gaylord, 1903) On the basis of subsequent findings, telangiectatic osteosarcoma was considered a variant of osteosarcoma (Ewing, 1922, 1939) Histopathology and Molecular Pathology of Bone and Extraskeletal Osteosarcomas 7 It is rare, less than 4% of all cases of osteosarcomas... multifocal osteosarcomas have been reported (Ackermann, 1948, Amstutz, 1969, Laurent et al, 1973) According to the mode of growth, osteosarcomas are subdivided into intramedullary and surface osteosarcomas Intramedullary osteosarcomas are further subdivided into typical intramedullary, telangiectatic and highly differentiated Surface osteosarcomas are subdivided into periosteal and parosteal osteosarcomas... treatment can alter tumour appearance In addition, osteosarcoma may be of any histologic grade Some contain highly pleomorphic cells and abundant mitotic figures, whereas others may be difficult to differentiate from benign neoplasms 6 Osteosarcoma 2.1.1 Osteoblastic osteosarcoma Conventional osteosarcoma is usually of the osteoblastic type In osteoblastic osteosarcoma the predominant matrix is bone and/or... benign tumour or non-bone forming malignancy (Cahan et al, 1948) Histologically, high grade osteosarcomas predominate Many of the reported cases of osteosarcoma arising in fibrous dysplasia have also been complicated by radiation therapy 2.5.3 Osteosarcomas in other benign precursors Other rare instances of secondary osteosarcomas have included cases arising in association with bone infarcts, endoprostheses... approach, based principally on histologic classification Under this system, central low grade osteosarcoma and parosteal osteosarcoma are considered Grade 1 sarcomas, with periosteal osteosarcoma considered Grade 2, and all other osteosarcomas considered Grade 3 (Dorfman et al, 1998, 2002) The exact cause of osteosarcoma is unknown The best known causative association environmental risk factor is exposure... chondroblastic differentiation may be confused with periosteal osteosarcoma The degree of cytologic atypia is greater in high grade surface osteosarcoma and the tumours generally show larger spindle cell areas Finally, unlike dedifferentiated parosteal osteosarcomas, low grade regions are not found in high grade surface osteosarcomas 12 Osteosarcoma Cortical destruction and invasion into the medullary... techniques have a limited role in diagnosing osteosarcoma, as the tumour is largely recognized by its morphologic features Because of the many varieties of osteosarcoma, diverse tumours are considered in its differential diagnosis 16 Osteosarcoma Currently, immunohistochemistry has limited application in the differential diagnosis of primary bone tumors In general, osteosarcoma has a broad immunoprofile . OSTEOSARCOMA Edited by Manish Agarwal Osteosarcoma Edited by Manish Agarwal Published by InTech Janeza. orders@intechopen.com Osteosarcoma, Edited by Manish Agarwal p. cm. ISBN 978-953-51-0506-0 Contents Preface VII Part 1 Osteosarcoma Workup