Book © 2022

Hardcover  ISBN 978-3-030-92070-8

Edition Number 2

Number of Pages X, 609

Number of Illustrations 12 b/w illustrations, 88 illustrations in colour

Topics Oncology, Pediatrics, Surgery

• Explains the differential diagnostic assessment appropriate at each tumor site

• Enables the clinician to take rare entities into account

• Provides detailed therapeutic guidelines for each of the rare tumors discussed

About this book

This is the first book to be devoted exclusively to rare tumors in children and adolescents.

The completely revised and updated second edition reflects the significant progress that has been accomplished as a result of intensified international networking, deeper implementation of novel diagnostic tools, and the advent of molecular targeted therapies.

Readers will find practical guidance on all aspects of clinical management, including diagnostic workup, multimodal therapy, follow-up, and management of adverse effects.

The discussion of differential diagnosis encompasses both frequent and rare tumor types, enabling clinicians to take rare entities into account during diagnostic assessment of childhood tumors.

Detailed therapeutic recommendations, developed in an international consensus process, are provided for specific rare tumors. In addition, general issues such as epidemiology, etiology, risk factors, biology and genetics, early detection, and screening are fully covered.

The book is written by an international and multidisciplinary group of specialists and will be an important compendium for all pediatric oncologists who care for patients with rare tumors.

Table of contents (47 chapters)

Front Matter

Pages i-x

Introduction

Front Matter

Pages 1-1

Rare Tumors: A Different Perspective on Oncology

Thomas A. Olson, Dominik T. Schneider, Ines B. Brecht, Andrea Ferrari

Pages 3-12

Epidemiology and Etiology of Rare Cancers

Front Matter

Pages 13-13

Epidemiology

Ines B. Brecht, Peter Kaatsch, Annalisa Trama

Pages 15-23

Biology and Etiology of Rare Pediatric Tumors

Ines B. Brecht, H. M. J. Merks

Pages 25-30

Childhood Cancer Predisposition

Johannes H. M. Merks, Illja Diets, Ines B. Brecht, Marjolijn CJ Jongmans

Pages 31-36

National and International Study Groups

Front Matter

Pages 37-37

National Initiatives in Europe

Andrea Ferrari, Daniel Orbach, Yves Reguerre, Ines B. Brecht, Dominik T. Schneider, Jan Godzinski et al.

Pages 39-48

EXPeRT: The European Cooperative Study Group for Pediatric Rare Tumors

Andrea Ferrari, Gianni Bisogno, Dominik T. Schneider

Pages 49-54

The United States

Farzana Pashankar, Carlos Rodriguez-Galindo

Pages 55-58

Rare Tumors of the Head and Neck

Front Matter

Pages 59-59

Epidemiology of Head and Neck Tumors

Jan Godzinski, Ines B. Brecht

Pages 61-62

Thyroid Carcinomas

Maura Massimino, Antje Redlich, Paola Collini, Peter Vorwerk

Pages 63-77

Nasopharyngeal Carcinoma

Udo Kontny, Carlos Rodriguez-Galindo, Daniel Orbach, Michela Casanova

Pages 79-97

Esthesioneuroblastoma

Barbara Hero, Gianni Bisogno, Dominik T. Schneider

Pages 99-106

Germ Cell Tumors of the Head and Neck

Dominik T. Schneider, Thomas A. Olson

Pages 107-111

Odontogenic Tumors

Jan Godzinski, Roberto Bianchi, Marco Guzzo

Pages 113-117

Salivary Gland Tumors

Marco Guzzo, Jan Godzinski

Pages 119-126

Laryngeal Tumors

Roberto Bianchi, Jan Godzinski, Marco Guzzo

Pages 127-133

Rare Tumors of the Head and Neck

Front Matter

Pages 135-135

Pathological Aspects of Mediastinal Tumors in Children and Adolescents

Alexander Marx, Claudia Spix, Christian Vokuhl

Pages 137-148

Mediastinal Germ Cell Tumors

Dominik T. Schneider, Giovanni Cecchetto, Thomas A. Olson

Pages 149-157

Pleuropulmonary Blastoma

Kris Ann P. Schultz, Yoav Messinger

Pages 159-167

Bronchial Carcinoids and Carcinomas

Dominik T. Schneider, Antje Redlich, Peter Vorwerk

Pages 169-176

Pediatric Pleural Mesothelioma

Nicolas André, Sergey K. V. V. Kashanskiy, Abbas Agaimy

Pages 177-184

Thymoma and Thymic Carcinoma in Children and Adolescents

Teresa Stachowicz-Stencel, Daniel Orbach, Jan Godzinski, Anna Synakiewicz

Pages 185-192

NUT Carcinoma

Christopher A. French

Pages 193-204

Rare Tumors of the Gastrointestinal Tract

Front Matter

Pages 205-205

Pathology and Differential Diagnosis

Bahig M. Shehata, Sarah S. Kappa

Pages 207-216

Tumors of the Esophagus and the Stomach

Dietrich von Schweinitz

Pages 217-222

Tumors of the Small Intestine, Colon, and Rectum

Stefano Signoroni, Maria Di Bartolomeo, Andrea Ferrari

Pages 223-244

Neuroendocrine Tumors (Carcinoids) of the Appendix

1. Redlich, M. Kuhlen, P. Vorwerk

Pages 245-247

Gastrointestinal Stromal Tumors in Children and Adolescents

1. Benesch, P. Reichardt

Pages 249-252

Pancreatic Tumors

Ewa Bien, Winfried Barthlen, Ines B. Brecht, Bence Sipos

Pages 253-278

Liver Tumors

Howard M. Katzenstein, Michela Casanova

Pages 279-289

Paediatric Peritoneal Mesothelioma

Nicolas André, Abbas Agaimy

Pages 291-298

Germ Cell Tumors and Genitourinary Tumors

Front Matter

Pages 299-299

Gonadal and Extragonadal Germ Cell Tumors, Sex Cord Stromal and Rare Gonadal Tumors

Dominik T. Schneider, Monica Terenziani, Giovanni Cecchetto, Thomas A. Olson

Pages 301-389

Rare Tumors of the Urinary Tract

Filippo Spreafico, Norbert Graf

Pages 391-417

Germ Cell Tumors and Genitourinary Tumors

Breast Cancer

Dominik T. Schneider

Pages 419-426

Rhabdoid Tumors

Front Matter

Pages 427-427

Extracranial Rhabdoid Tumours

Michael C. Frühwald, Franck Bourdeaut, Rhoikos Furtwängler

Pages 429-447

Rare Tumors of the Peripheral Nervous System

Front Matter

Pages 449-449

Rare Tumours of the Peripheral Nervous System: Intra-adrenal (Phaeochromocytoma) and Extra-adrenal Paraganglioma

Bernadette Brennan

Pages 451-457

Adrenocortical Tumors in Children

Carlos Rodriguez-Galindo

Pages 459-471

Medulloepithelioma

Gianni Bisogno

Pages 473-475

Chordoma

Gianni Bisogno

Pages 477-480

Rare Tumors of the Skin and Subcutaneous Tissue

Front Matter

Pages 481-481

Epidemiology and Differential Diagnosis of Cutaneous Tumors

Andrea Ferrari, Paola Collini

Pages 483-485

Cutaneous Melanoma

Alice Indini, Alberto S. Pappo, Thomas K. Eigentler, Paola Collini, Andrea Ferrari

Pages 487-510

Skin Carcinoma

Thomas K. Eigentler, Andrea Ferrari

Pages 511-512

Other Rare Tumors of the Skin and Subcutaneous Tissue

Alberto Pappo, Andrea Ferrari

Pages 513-516

Vascular Tumors

Front Matter

Pages 517-517

Vascular Tumors Including Kaposi Sarcoma

Jochen Rössler

Pages 519-532

Rare Mesenchymal Tumors

Front Matter

Pages 533-533

Soft Tissue Sarcomas

Andrea Ferrari, Rita Alaggio

Pages 535-582

Rare Bone Tumors

Uta Dirksen, Andrea Ferrari

Pages 583-589

Tumors of Unknown Primary Site

Front Matter

Pages 591-591

Tumors of Unknown Primary Site

Thomas A. Olson

Pages 593-598

Rare Tumors as Second Malignancies

Front Matter

Pages 599-599

Rare Subsequent Primary Cancers in Pediatric Cancer Survivors

Ann C. Mertens, Thorsten Langer

Pages 601-609

Keywords

Rare tumors of the head and neck

Thyroid carcinomas

Nasopharyngeal carcinoma

Rare tumors of the gastrointestinal tract

Germ cell tumors

Rhabdoid tumors

Vascular tumors

Rare mesenchymal tumors

Rare tumors as second malignancies

Rare tumors of the skin

Editors and Affiliations

Clinic of Pediatrics, Municipal Hospital Dortmund, University Witten-Herdecke, Dortmund, Germany

Dominik T. Schneider

Department of Hematology and Oncology, University Children’s Hospital, Tübingen, Germany

Ines B. Brecht

Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta and Emory University, Atlanta, USA

Thomas A. Olson

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy

Andrea Ferrari

About the editors

Dominik T. Schneider, MD, is Director of the Clinic of Pediatrics at Dortmund Municipal Hospital and an extracurricular Professor of Pediatrics at Heinrich Heine University in Düsseldorf, Germany. After graduating from medical school at the Charité in Berlin, he completed his clinical training at Heinrich Heine University and also worked as a post doc research fellow at Johns Hopkins University in Baltimore. Dr. Schneider is vice-chair of the German MAKEI working group and since 2006 has co-chaired the German Working Group and Registry of Pediatric Rare Tumors (STEP). He is involved in several clinical research groups, including the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) and the Ovarian and Testicular Sex Cord Tumor Registry.  He has served as an executive board member of the German Society of Pediatric Oncology and Hematology (GPOH) and is a member of the executive board of the German Society of Pediatrics (DGKJ). His research focus is on germ cell tumors and rare tumors, in particular rare gonadal tumors.

Ines B. Brecht, MD, is a senior physician and pediatric oncologist at the Children’s University Hospital Tübingen, Germany. She completed her medical studies at Heinrich Heine University, Düsseldorf, in 2000 and undertook clinical training in Ulm, Erlangen, and Stuttgart before completing a post doc research fellowship at the Children’s Hospital of Michigan, Wayne State University (Detroit, USA). Dr. Brecht has received scholarships from the German Academic Scholarship Foundation, the Alfried Krupp von Bohlen and Halbach Foundation, and the Madeleine Schickedanz Foundation.  Since 2006 she has co-chaired the German Working Group and Registry of Pediatric Rare Tumors (STEP). Dr. Brecht is a co-founder of the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) and chair of the group since 2021. In 2017 she founded the Center for Rare Pediatric Tumors in Tübingen. Dr. Brecht is involved in clinical research on several rare pediatric tumors, especially on melanomas, colorectal carcinomas, and the epidemiology and genetics of rare pediatric cancers.

Andrea Ferrari, MD, is an oncology consultant and responsbile of the Outpatient Service at the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan in Italy, a position he has held since 1994. He tackled research projects dedicated to soft tissue sarcomas, pediatric rare tumors and adolescents/young adults projects. He worked as chair and board member of various international cooperative groups, developing a large experience in leading and coordinating multidisciplinary working team. Among the various accomplishments, he co-founded the Italian TREP project dedicated to pediatric very rare tumors, and he co-founded the European EXPERT (European Cooperative Study Group for Pediatric Rare Tumors) project, for which he has been the first chair.

He was founding member of the EpSSG (European Pediatric Soft Tissue Sarcoma Study Group). Within EpSSG, he is member of the board and chairman of the NRSTS (Non-Rhabdomyosarcoma Soft Tissue Sarcoma Scientific Committee). In the last years, Dr. Ferrari dedicated himself also to the special needs of adolescents and young adults (AYA) with cancer. He developed the “Youth Project” in his center. He currently chairs the Italian Committee on AYA, and the AYA Committee of the SIOPE (European Society for Paediatric Oncology), and he is co-chair of the joint Working Group on AYA of SIOPE and ESMO (European Society of Medical Oncology). He is author or co-author of more than 450 papers published on international scientific journals, and 30 book chapters.

Thomas A. Olson, MD, is Professor of Pediatrics at Emory University School of Medicine, Atlanta, Georgia, and Program Director Solid Tumor at the Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta.  He is also Chair of the Protocol Review and Monitoring Committee of the Winship Cancer Institute, Emory University School of Medicine. Dr. Olson graduated from Loyola University Chicago Stritch School of Medicine, Maywood, Illinois and did his residency and fellowship training at Walter Reed Army Medical Center, Washington, DC.  As a member of the Children’s Oncology Group, he has served on the Rare Tumor Executive Committee as Chair of the Germ Cell Tumor Subcommittee.  He was a founding member of and continues to serve on the Malignant Germ Cell Tumor International Consortium.  He is an editorial board member of the National Cancer Institute’s PDQ Pediatric Treatment and Journal of Clinical Oncology. He has authored numerous articles and chapters on germ cell tumors and rare tumors.