Table Of ContentPaul 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
