Rare Tumors in Children and Adolescents PDF
Preview Rare Tumors in Children and Adolescents
Pediatric Oncology Series Editors: Gregory H. Reaman · Franklin O. Smith Dominik T. Schneider Ines B. Brecht Thomas A. Olson Andrea Ferrari Editors Rare Tumors in Children and Adolescents Second Edition Pediatric Oncology Series Editors Gregory H. Reaman Center for Drug Evaluation and Research U.S. Food and Drug Administration Silver Spring, MD, USA Franklin O. Smith Medpace Cincinnati, OH, USA This series provides up-to-date information on important topics in pediatric oncology, from the diagnosis and treatment of particular forms of disease through to, for example, radiation oncology, supportive care, and survivorship. The entire spectrum of clinical management is covered with the aim of equipping readers with the latest knowledge relevant to daily practice. In addition, clinical, methodological, and research issues are addressed. The series benefits from homogeneous design and consistently high quality of illustrations. The volume editors are internationally renowned authorities and contributing authors have been selected for their expertise in the subjects discussed. Pediatric Oncology will serve pediatric oncologists, fellows, and residents both as a comprehensive source of information and as a quick reference. The series will also be of interest to pediatricians and general practitioners. More information about this series at https://link.springer.com/bookseries/5421 Dominik T. Schneider Ines B. Brecht • Thomas A. Olson Andrea Ferrari Editors Rare Tumors in Children and Adolescents Second Edition Editors Dominik T. Schneider Ines B. Brecht Clinic of Pediatrics Department of Hematology and Municipal Hospital Dortmund, Oncology University Witten-Herdecke University Children’s Hospital Dortmund, Germany Tübingen, Baden-Württemberg, Germany Thomas A. Olson Andrea Ferrari Aflac Cancer and Blood Disorders Pediatric Oncology Unit Center Fondazione IRCCS Istituto Nazionale Children’s Healthcare of Atlanta and dei Tumori Emory University Milano, MI, Italy Atlanta, GA, USA ISSN 1613-5318 ISSN 2191-0812 (electronic) Pediatric Oncology ISBN 978-3-030-92070-8 ISBN 978-3-030-92071-5 (eBook) https://doi.org/10.1007/978-3-030-92071-5 © Springer Nature Switzerland AG 2022 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland Preface If you work on frequent cancer, do randomized trials. If you work on rare cancers—FIND FRIENDS! All pediatric cancers are rare events when viewed in the backdrop of all cancers. And within the scope of childhood cancers, there are several infre- quent tumors that pediatric oncologists would classify as “rare pediatric tumors.” Therefore, what is the point in working on a book that specifically focuses on cancers that are “almost never” diagnosed? The most important reasons may be the child who suffers from a specific tumor, the families of these children not knowing how to cope with these diagnoses, and the treating physicians who may be confronted with a tumor, for which no evidence- based recommendations are available. Thus, this book shall help to take a group of tumors into focus, which otherwise would remain orphan diseases. In fact, rare cancers as a group are not as uncommon as their designation may suggest. They contribute to at least 5% of all childhood cancers. However, caring for children with such rare cancers requires a tremendous effort, pri- marily because sufficient information on diagnosis and therapy is missing. As a consequence, the effort for treating physicians is inadequately high. Advances in pediatric oncology have always been facilitated through sharing information and networking between experts. Networks first began among groups of institutions. Later, networks were developed on a national basis, fostered by national cooperative groups. Recently, more and more interna- tional pediatric collaborations have been established to advance prospective therapeutic trials for “more common pediatric cancers.” However, since rare tumors present with extremely low incidence, international collaboration is even more essential for these patients. Otherwise, each patient with a rare tumor will remain a “first patient” that cannot benefit from experience gath- ered from other patients with the same diagnosis. This book attempts to fill this information gap, by providing pediatricians, pediatric oncologists, radiotherapists, and pediatric surgeons all currently available information required for diagnostic assessment and therapy of such patients. The second edition includes detailed checklists for diagnostic proce- dures and updated information on multimodal therapy of rare cancers. Furthermore, it integrates up-to-date information that has emerged from recent molecular genetic studies. These have often contributed to more pre- cise classification and better understanding of the tumor biology, and some- times even offered new targeted therapeutic strategies. v vi Preface For most chapters, authors from different national study groups have shared their knowledge and developed common recommendations. Thus, the book reflects the growing experience gained through international collabora- tion. As for some entities, consensus recommendations have recently been developed; these are also integrated into the updated chapters. For other enti- ties, these chapters represent the first comprehensive review in this particular entity to date. Sometimes, this has been a slow and stepwise but finally suc- cessful process. The discussions have also provided a fruitful and fantastic learning experience. We hope this book may provide a framework for future evolution into internationally accepted guidelines. Finally, this book is also the result of better understanding, deeper collaboration, and growing friend- ship—because: it is networking, or not working. Therefore, we would like to thank all authors for their tremendous effort in writing their chapters. We would also like to thank Springer for the opportu- nity to further develop this project. Last, we thank our families for their con- tinuous and loving support and their patience. Encouraged by the overwhelming feedback to the first edition of our book, we sincerely hope that this book will again find the interest of the interna- tional audience and will be taken to hand often, rather than rarely. Dortmund, Germany Dominik T. Schneider Tübingen, Germany Ines B. Brecht Atlanta, GA, USA Thomas A. Olson Milano, Italy Andrea Ferrari Contents Part I Introduction 1 Rare Tumors: A Different Perspective on Oncology . . . . . . . . . . 3 Thomas A. Olson, Dominik T. Schneider, Ines B. Brecht, and Andrea Ferrari Part II E pidemiology and Etiology of Rare Cancers 2 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Ines B. Brecht, Peter Kaatsch, and Annalisa Trama 3 Biology and Etiology of Rare Pediatric Tumors . . . . . . . . . . . . . . 25 Ines B. Brecht and H. M. J. Merks 4 Childhood Cancer Predisposition . . . . . . . . . . . . . . . . . . . . . . . . . 31 Johannes H. M. Merks, Illja Diets, Ines B. Brecht, and Marjolijn CJ Jongmans Part III N ational and International Study Groups 5 National Initiatives in Europe . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 Andrea Ferrari, Daniel Orbach, Yves Reguerre, Ines B. Brecht, Dominik T. Schneider, Jan Godzinski, and Bernadette Brennan 6 EXPeRT: The European Cooperative Study Group for Pediatric Rare Tumors . . . . . . . . . . . . . . . . . . . . . . . . . 49 Andrea Ferrari, Gianni Bisogno, and Dominik T. Schneider 7 The United States . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Farzana Pashankar and Carlos Rodriguez-Galindo Part IV R are Tumors of the Head and Neck 8 Epidemiology of Head and Neck Tumors . . . . . . . . . . . . . . . . . . . 61 Jan Godzinski and Ines B. Brecht 9 Thyroid Carcinomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 Maura Massimino, Antje Redlich, Paola Collini, and Peter Vorwerk vii viii Contents 10 Nasopharyngeal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 Udo Kontny, Carlos Rodriguez-Galindo, Daniel Orbach, and Michela Casanova 11 Esthesioneuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 Barbara Hero, Gianni Bisogno, and Dominik T. Schneider 12 Germ Cell Tumors of the Head and Neck . . . . . . . . . . . . . . . . . . . 107 Dominik T. Schneider and Thomas A. Olson 13 Odontogenic Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 Jan Godzinski, Roberto Bianchi, and Marco Guzzo 14 Salivary Gland Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119 Marco Guzzo and Jan Godzinski 15 Laryngeal Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 Roberto Bianchi, Jan Godzinski, and Marco Guzzo Part V Rare Tumors of the Head and Neck 16 Pathological Aspects of Mediastinal Tumors in Children and Adolescents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137 Alexander Marx, Claudia Spix, and Christian Vokuhl 17 Mediastinal Germ Cell Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Dominik T. Schneider, Giovanni Cecchetto, and Thomas A. Olson 18 Pleuropulmonary Blastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159 Kris Ann P. Schultz and Yoav Messinger 19 Bronchial Carcinoids and Carcinomas . . . . . . . . . . . . . . . . . . . . . 169 Dominik T. Schneider, Antje Redlich, and Peter Vorwerk 20 Pediatric Pleural Mesothelioma . . . . . . . . . . . . . . . . . . . . . . . . . . . 177 Nicolas André, Sergey K. V. V. Kashanskiy, and Abbas Agaimy 21 Thymoma and Thymic Carcinoma in Children and Adolescents 185 Teresa Stachowicz-Stencel, Daniel Orbach, Jan Godzinski, and Anna Synakiewicz 22 NUT Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193 Christopher A. French Part VI R are Tumors of the Gastrointestinal Tract 23 Pathology and Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . 207 Bahig M. Shehata and Sarah S. Kappa 24 Tumors of the Esophagus and the Stomach . . . . . . . . . . . . . . . . . 217 Dietrich von Schweinitz Contents ix 25 Tumors of the Small Intestine, Colon, and Rectum . . . . . . . . . . . 223 Stefano Signoroni, Maria Di Bartolomeo, and Andrea Ferrari 26 Neuroendocrine Tumors (Carcinoids) of the Appendix . . . . . . . . 245 A. Redlich, M. Kuhlen, and P. Vorwerk 27 Gastrointestinal Stromal Tumors in Children and Adolescents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 249 M. Benesch and P. Reichardt 28 Pancreatic Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Ewa Bien, Winfried Barthlen, Ines B. Brecht, and Bence Sipos 29 Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279 Howard M. Katzenstein and Michela Casanova 30 Paediatric Peritoneal Mesothelioma . . . . . . . . . . . . . . . . . . . . . . . 291 Nicolas André and Abbas Agaimy Part VII Germ Cell Tumors and Genitourinary Tumors 31 Gonadal and Extragonadal Germ Cell Tumors, Sex Cord Stromal and Rare Gonadal Tumors . . . . . . . . . . . . . . . 301 Dominik T. Schneider, Monica Terenziani, Giovanni Cecchetto, and Thomas A. Olson 32 Rare Tumors of the Urinary Tract. . . . . . . . . . . . . . . . . . . . . . . . . 391 Filippo Spreafico and Norbert Graf 33 Breast Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419 Dominik T. Schneider Part VIII Rhabdoid Tumors 34 Extracranial Rhabdoid Tumours . . . . . . . . . . . . . . . . . . . . . . . . . . 429 Michael C. Frühwald, Franck Bourdeaut, and Rhoikos Furtwängler Part IX R are Tumors of the Peripheral Nervous System 35 Rare Tumours of the Peripheral Nervous System: Intra-adrenal (Phaeochromocytoma) and Extra- adrenal Paraganglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 451 Bernadette Brennan 36 Adrenocortical Tumors in Children . . . . . . . . . . . . . . . . . . . . . . . 459 Carlos Rodriguez-Galindo 37 Medulloepithelioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473 Gianni Bisogno
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