Pediatric Oncology: A Comprehensive Guide PDF
Preview Pediatric Oncology: A Comprehensive Guide
Paul Imbach Thomas Kühne Robert J. Arceci Editors Pediatric Oncology A Comprehensive Guide Third Edition 123 Pediatric Oncology Paul Imbach (cid:129) Thomas Kühne Robert J. Arceci Editors Pediatric Oncology A Comprehensive Guide Third Edition Editors Paul Imbach, MD, Emeritus Robert J. Arceci, MD, PhD, FAAP Department of Pediatrics Department of Hematology/Oncology Medical Faculty of the University of Basel Children’s Center for Cancer and Blood Basel Disorders, The Ron Matricaria Institute Switzerland of Molecular Medicine, Phoenix Children’s Hospital, University of Arizona College Thomas Kühne, MD of Medicine – Phoenix, Department of Pediatrics Phoenix, AZ Pediatric Oncology-Hematology USA University Children’s Hospital Basel Basel Switzerland ISBN 978-3-319-06009-5 ISBN 978-3-319-06010-1 (eBook) DOI 10.1007/978-3-319-06010-1 Springer Cham Heidelberg New York Dordrecht London L ibrary of Congress Control Number: 2014942374 © Springer International Publishing Switzerland 2014 T his work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfi lms 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. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifi cally for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher's location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. T he use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specifi c statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com) Foreword H ardly any fi eld of pediatrics refl ects the medical advances of the past three decades as dramatically as pediatric oncology. Forty years ago, when I began my pediatric training, three quarters of all children with malignancies died of their disease. Today 80 % are healed. Three reasons for this can be delineated. First, therapy optimiza- tion studies have led to constant improvement through adaptation of treatment to individual cases. Second, new drugs, new combinations and new dosages have been developed and tolerance to therapy has been improved by supportive measures. Finally and with unexpected rapidity, molecular biology and molecular genetics have revolutionized hematology and oncology. Molecular mechanisms causing malignant tumor cell growth are being identifi ed and used for therapeutic decision- making. We cannot yet – with certain exceptions – intervene specifi cally in the aberrant regulation of malignant growth, but the foundations have been laid. P ediatric oncology is, rightly, viewed as a clinical and scientifi c subspecialty of pediatrics. This does not mean it is not of interest to the general pediatrician or spe- cialists in other areas of pediatrics. On the contrary, in the early stages of a malig- nant disease the symptoms are often nonspecifi c. Although one may primarily suspect a tumor or leukemia, other diseases cannot be completely excluded. Conversely, the vague general symptoms that can be caused by a malignancy may lead themselves to misinterpretation. Furthermore, a team is required to care for the patients: pediatricians, pediatric or specialist surgeons, specialized nurses, psychol- ogists, social workers and chaplains. Pediatric oncology is holistic, integrated medi- cine in the true sense of the word. And with regard to medical training, nowhere in pediatrics can one gain a closer experience with treatment of infections and other particular topics than in pediatric oncology. Oncology has an undisputed place in the training of every pediatrician. Equally, comprehensive general pediatric training is important for every future pediatric hematologist/ocologist. T here are a number of excellent, exhaustive textbooks on oncology that are indis- pensable in training. However, there is also a need for a compact guide offering rapid orientation in the situations encountered by all who work in pediatric oncol- ogy. Precisely, that is provided by this book by Paul Imbach, Thomas Kühne and Robert J. Arceci. I wish them the success they deserve. Innsbruck , Austria Gerhard Gaedicke 2013 v Pref ace T he healing process in children and adolescents with oncological diseases depends greatly on the knowledge and experience of all those involved in the patients’ care: physicians, specialist nurses, psycho-oncologists and others. This last group embraces parents, siblings and teachers as well as laboratory staff, physiotherapists, chaplains, social workers and other hospital personnel. Increasingly, the patients’ general practitioners and pediatricians and external nurses are also becoming involved. Knowledge and experience on the part of caregivers are necessary for a complete picture of the patient, who is thus enabled to play a full part in his or her own healthcare: the power of the informed patient. Similarly, the family becomes an integral part of the overall care of pediatric patients. Whether a young patient is waiting for the diagnosis, undergoing intensive therapy, or suffering a complication or setback, whether he/she knows that the disease has almost certainly been healed or that it is progressing with early death as the probable consequence – in every situ- ation, full information is the basis of optimal care. T his book was written to improve the fundamental dissemination of knowledge. It has no pretensions to replace the standard textbooks and the journals regarding pediatric oncology. In this third edition the chapters systematically describe the various disease groups. The content is adapted by new fi ndings within the last 3 years. Some of these chapters were written by Thomas Kühne, for many years my trusted col- league. He added a new chapter on “Rare Tumors” (Chap. 1 8 ). Robert Arceci of Phoenix Children’s Hospital and the University of Arizona, editor-in-chief of Pediatric Blood and Cancer, brought his vast experience to bear on the English translation and wrote Chap. 8 on Histiocytoses. Chapter 2 0 has been rewritten by the nurse Annette Schneider working at the pediatric oncology department at the University Children’s Hospital in Basel for many years, since the former writers have new positions as leaders in public health and pediatrics. My heartfelt thanks go to all of the contributing authors and to the responsible staff at Springer Heidelberg for their commitment to this project. M ay this book help to create an atmosphere of trust, hope and joy in the face of potentially life-threatening disease. Basel, Switzerland Paul Imbach , MD, Emeritus 2005/2011/2014 vii Abbreviations aCML Atypical chronic myeloid leukemia ADH Antidiuretic hormone AFP α-Fetoprotein ALCL Anaplastic large cell lymphoma ALK Anaplastic lymphoma kinase ALL Acute lymphoblastic leukemia ALPS Autoimmune lymphoproliferative syndrome AMCL Acute monocytic leukemia AMKL Acute megakaryocytic leukemia AML Acute myelogenous leukemia AMML Acute myelomonocytic leukemia ANAE α-Naphthyl acetate esterase ANC Absolute neutrophil count APL Acute promyelocytic leukemia ATRA All transretinoid acid b-HCG β-Choriogonadotropin BL Burkitt lymphoma BLL Burkitt-like lymphoma BWS Beckwith–Wiedemann syndrome CD Cluster determination CEL Chronic eosinophilic leukemia CML Chronic myelogenous leukemia CMML Chronic myelomonocytic leukemia CNL Chronic neutrophilic leukemia CNS Central nervous system COG Cooperative oncology group CT Computed tomography CTL Cytotoxic T-lymphocytes CVID Common variable immune defi ciency DI DNA index DIC Disseminated intravascular coagulation DLI Donor lymphocyte infusion DNA Deoxynuclein acid EBV Epstein–Barr virus ix
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