Autor: Giorgio Perilongo, Arthur Zimmermann, Dietrich von Schweinitz, Marcio Malogolowkin
Herausgeber: Arthur Zimmermann, Giorgio Perilongo
Titel: Pediatric Liver Tumors
Verlag: Springer-Verlag
ISBN/ISSN: 9783642145049
Auflage: 1
Preis : CHF 160.10
Kategorie: Medizin & Pharmazie
Sprache: English
Technische Daten
Seiten: 232
Kopierschutz: DRM
Geräte: PC/MAC/eReader/Tablet
Formate: PDF
The field of liver tumors in children has seen tremendous therapeutic advances over recent years. This has been achieved through a much better understanding of the biology of disease, improved diagnostic procedures, refined methods of pretreatment tumor staging, the implementation of highly efficient chemotherapy and surgery, detailed monitoring of toxicity, and careful follow-up strategies. International controlled trials have played a key role in these advances, and many leading figures in the trials are among the editors and authors of this book. Their principal goal in Hepatic Tumors in Children is to provide the reader with a complete update on this complex and rapidly evolving field. All aspects of an impressive success story are covered, disclosing how the outcome of a previously devastating disease has been so dramatically improved. This book will prove essential reading for all who are involved in the care of children with liver tumors.

Prof.Dr.med.em Arthur Zimmermann graduated in Medicine at the University of Berne, Switzerland. He attended the local Pathology Residency Program, followed by several years of experipmental basic research in the fields of cancer cell cycle mutants and mechanisms of leukocyte and cancer cell motility. As chief of staff and then head of the division of surgical pathology at the University of Berne he specialized in hepatobiliary and pancreatic tumor pathology, where he became the author and coauthor of more than 500 publications. Until July 2009, Prof. Zimmermann was Acting Director of the Institute of Pathology of the University of Berne. As an expert in pediatric liver tumors he is an international consultant and holds the Pathology Review Center for the International Childhood Liver Tumor Study Group ( SIOPEL ). Prof. Giorgio Perilongo graduated in Medicine in 1980 at the University of Padua, Italy; he then attended the local Pediatric Residency program for then completing his post-graduate education with a fellowship in Hematology-Oncology first and in Neuro-Oncology thereafter at the Children's Hospital of Philadelphia. Presently he is the Chairman of the Department of Pediatrics of the University Hospital in Padua. He was among the founders of the International Childhood Liver Tumor Study Groups (otherwise known as SIOPEL group), the international cooperative research group on childhood liver tumors based in Europe conceived within the umbrella of the International Society of Pediatric Oncology. Within this group is has and he is still playing a very active role. He chaired a series of clinical trials already published in the main international scientific journals.
Pediatric Oncology1
Copyright Page 3
1: Introduction11
2: Historical Background12
2.1 Introduction12
2.2 The Dark Ages13
2.3 Advent of Printing13
2.4 Early Childrens Hospital13
2.5 Pioneer Pathologists and/or Their Textbooks14
2.6 Pioneers in Surgical Anatomy and Liver Surgery14
2.7 Liver Transplantation14
2.8 International Oncology Groups15
2.8.1 COG15
2.8.2 SIOP15
2.8.3 SIOPEL (SIOP Epithelial Liver tumors)15
2.8.4 IPSO16
2.8.5 SIOP ASIA16
2.8.6 PODC (Pediatric Oncology in Developing Countries)16
2.8.7 EONS (European Oncology Nursing Specialists)16
2.9 National Pediatric Oncology Societies16
2.10 Clinical Liver Trials17
2.11 Conclusions and Future Outlook17
3: Epidemiology of Pediatric Liver Tumors24
3.1 Introduction24
3.2 Descriptive Epidemiology of Pediatric Liver Tumors25
3.2.1 Spectrum and Frequency of Liver Tumors in Children25
3.2.2 International Variations in Incidence25
3.2.3 Time Trends in Incidence27
3.3 Analytical Epidemiology of Hepatoblastoma and Hepatocellular Carcinoma in Children27
3.3.1 Overview of Environmental Risk Factors27
3.3.2 Very Low Birth Weight and Hepatoblastoma28
3.3.3 Parental Tobacco Smoking and Hepatoblastoma29
3.3.4 Associations with Heritable Cancer Predisposition, Congenital Malformation Syndromes, and Anomalies29
3.3.5 Etiology of Hepatocellular Carcinoma in Children30
3.4 Implications of Findings from Analytical Studies of Pediatric Liver Tumors31
3.5 Conclusions32
4: Molecular Aspects of Hepatoblastoma36
4.1 Introduction36
4.2 Basics of Hepatoblastoma36
4.3 Familial Forms of Hepatoblastoma37
4.5 Altered Developmental Signaling Pathways in Hepatoblastoma40
4.5.1 The Wnt Signaling Pathway40
4.5.2 The Hedgehog Signaling Pathway42
4.5.3 The Insulin-Like Growth Factor Axis43
4.5.4 The Hepatocyte Growth Factor/c-Met Pathway44
4.6 Epigenetically Altered Tumor Suppressor Genes in Hepatoblastoma45
4.7 Gene Signatures as Predictors of Outcome46
5: Ontogenetic Aspects of Liver Tumors52
5.1 Introduction52
5.2 Liver Ontogenesis: Pathways from Endoderm to Liver52
5.2.1 Generation of the Hepatoblast and Hepatocyte Lineages52
5.2.2 Development of the Cholangiocyte Lineage and the Bile Duct System53
5.3 Stem Cells in the Pathogenesis of Hepatoblastomas54
5.4 Undifferentiated Epithelial Hepatoblastomas and the Rhabdoi