The Hereditary Basis Of Childhood Cancer

This volume elaborates on the research and clinical implications of the hereditary and molecular basis of childhood cancers. The focus of the 'disease-related' chapters of the book is to integrate what is known about the molecular basis of that particular clinical entity (or group of related entities) with the clinical manifestations, to relate the relationship of the molecular oncologic pathways with relevant developmental or non-human species biology in order to better understand the complexity of these systems. The resulting clinical implications of understanding this biology are elaborated on. Chapters 13-16 discuss the broader psychosocial, ethical and genetic counseling issues that arise and that are so critical to translating the knowledge gained from advances in molecular genetics into the clinic. Chapter 12 in particular provides a unique perspective of the application of this knowledge in less-developed nations where 'modern' technologies may not be readily available, but where the clinical manifestations of these disorders are prevalent.

This volume elaborates on the research and clinical implications of the hereditary and molecular basis of childhood cancers. The focus of the ‘disease-related’ chapters of the book is to integrate what is known about the molecular basis of that particular clinical entity (or group of related entities) with the clinical manifestations, to relate the relationship of the molecular oncologic pathways with relevant developmental or non-human species biology in order to better understand the complexity of these systems. The resulting clinical implications of understanding this biology are elaborated on. Chapters 13-16 discuss the broader psychosocial, ethical and genetic counseling issues that arise and that are so critical to translating the knowledge gained from advances in molecular genetics into the clinic. Chapter 12 in particular provides a unique perspective of the application of this knowledge in less-developed nations where ‘modern’ technologies may not be readily available, but where the clinical manifestations of these disorders are prevalent.

Get a quick, expert overview of the many key facets of pediatric cancer genetics with this concise, practical resource by Dr. Nathaniel H. Robin and Meagan Farmer, MS, CGC, MBA. Ideal for pediatric oncologists and all providers who care for children, this easy-to-read reference addresses the remarkable potential of genetic testing as well as the complexities of choosing the correct test, understanding the results, and counseling the family. Features a wealth of information on pediatric cancer genetics, including the epidemiology and biology of cancer and the genetic evaluation process and role of genetic counselors. Highlights examples of syndromes that present in childhood and increase susceptibility to cancer. Discusses the genetic evaluation process in context of the multidisciplinary care of children with cancer. Considers the ethical and legal issues of genetic testing in children and provides illustrative case examples. Consolidates today’s available information and guidance in this timely area into one convenient resource.

Cancer is the most common cause of disease-related death in children beyond the newborn period. Most cancers are thought to arise sporadically; however, classical studies of well-defined familial cancer associations, known as cancer predisposition syndromes (CPS), together with emerging work arising from new high-resolution genomic platforms have confirmed that at least 25% of childhood cancers result from hereditary factors. The spectrum of cancers found in the diverse array of known hereditary cancer syndromes is vast. Similarly, the number of genes linked to these syndromes continues to expand. This chapter explores the genotype:phenotype correlations in several defined cancer predisposition syndromes that primarily affect children. In particular, a selection of syndromes that are caused by germline mutations in classical tumor suppressor genes (RB1, TP53, WT1) and oncogenes (RET), syndromes associated with congenital developmental anomalies (Beckwith–Wiedemann syndrome, Gorlin syndrome) and an emerging syndrome associated with microRNA processing (DICER1) provide examples of the heterogeneity that these syndromes exhibit. The chapter concludes with a discussion of the clinical impact of genetic testing and clinical surveillance for early cancer detection.

It has been recognized for almost 200 years that certain families seem to inherit cancer. It is only in the past decade, however, that molecular genetics and epidemiology have combined to define the role of inheritance in cancer more clearly, and to identify some of the genes involved. The causative genes can be tracked through cancer-prone families via genetic linkage and positional cloning. Several of the genes discovered have subsequently been proved to play critical roles in normal growth and development. There are also implications for the families themselves in terms of genetic testing with its attendant dilemmas, if it is not clear that useful action will result. The chapters in The Genetics of Cancer illustrate what has already been achieved and take a critical look at the future directions of this research and its potential clinical applications.

This volume elaborates on the research and clinical implications of the hereditary and molecular basis of childhood cancers. The focus of the ‘disease-related’ chapters of the book is to integrate what is known about the molecular basis of that particular clinical entity (or group of related entities) with the clinical manifestations, to relate the relationship of the molecular oncologic pathways with relevant developmental or non-human species biology in order to better understand the complexity of these systems. The resulting clinical implications of understanding this biology are elaborated on. Chapters 13-16 discuss the broader psychosocial, ethical and genetic counseling issues that arise and that are so critical to translating the knowledge gained from advances in molecular genetics into the clinic. Chapter 12 in particular provides a unique perspective of the application of this knowledge in less-developed nations where ‘modern’ technologies may not be readily available, but where the clinical manifestations of these disorders are prevalent.

Cancer is the second commonest cause of death, after accidents, among children in developed countries, while in developing countries, improvements in the control of communicable diseases and the occurrence of premature delivery may lead to the emergence of cancer in children as a greater public health problem than in the past. The rationale for considering childhood cancers separately from cancers in adults in that there are differences in the sites of occurrence, in the histological appearance and in their clinical behavior. This book reviews the epidemiology of specific types of childhood cancer to mid-1997. The scale of the problem is first reviewed in a major compilation of data on the descriptive epidemiology of childhood cancer, then separate chapters describe studies of the different postulated causal factors and summarize what can be concluded from them.

This book provides information on a wide variety of issues ranging from genetics to clinical description of the syndromes, genetic testing and counseling, and clinical management including surveillance, surgical and prophylactic interventions, and chemoprevention. Moreover, current hot issues, such as the identification of novel causal genes and the challenges we face, and the relevance of cancer risk modifiers, both genetic and environmental, are also discussed. This reference book is great for geneticists, oncologists, genetic counselors, researchers, clinicians, surgeons and nurses dedicated to, or interested in, hereditary cancer. The best and most recognized experts in the field have contributed to this project, guaranteeing updated information, accuracy and the discussion of topical issues.