{"title":"Neuroblastoma","authors":"A. Eggert, G. Brodeur, G. Schleiermacher","doi":"10.1007/978-3-030-18396-7","DOIUrl":"https://doi.org/10.1007/978-3-030-18396-7","url":null,"abstract":"","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115617441","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Soft-Tissue Sarcomas","authors":"G. Bisogno, H. Merks","doi":"10.1093/MED/9780198797210.003.0025","DOIUrl":"https://doi.org/10.1093/MED/9780198797210.003.0025","url":null,"abstract":"This chapter discusses soft-tissue sarcomas (STS) in children and adolescents, which comprise approximately 8% of all paediatric malignancies. It highlights the clinical, pathological, and biological characteristics of this heterogeneous group of tumors derived from mesenchymal cells, and it describes the current management and the treatment results. The first part of the chapter is dedicated to the most frequent paediatric STS, rhabdomyosarcomas (RMS), while the second part covers the diagnosis and treatment strategies for non-rhabdomyosarcoma STS (NRSTS) including synovial sarcomas (SS), adult-type NRSTS, and other histotypes (infantile fibrosarcoma (IFS), desmoplastic small round-cell tumour (DSRCT), and malignant rhabdoid tumour (MRT)).","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"24 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116732382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Acute Lymphoblastic Leukaemia","authors":"D. Schewe, Mignon Loh, R. Pieters, M. Schrappe","doi":"10.1093/med/9780198797210.003.0015","DOIUrl":"https://doi.org/10.1093/med/9780198797210.003.0015","url":null,"abstract":"Acute lymphoblastic leukaemia (ALL) is the most common type of cancer in childhood. There have been continuous advances in the treatment of this disease in the last decades, mainly due to the unceasing development of cooperative international clinical trials. The identification of novel risk groups based on molecular genetic findings has added to improvements in risk stratification in many patients, but not in all. One of the main problems in paediatric ALL is relapsed and refractory disease requiring the evaluation of targeted agents and immunotherapies in clinical studies in children. Furthermore, the reduction of therapy intensity to avoid late adverse effects is a major focus in patients with low-risk disease. This chapter summarizes epidemiology, diagnostic modalities, prognostic factors, and therapy options, including allogeneic stem-cell transplantation, in paediatric ALL. It also focuses on controversial issues in specific patient subgroups defined by their age and molecular alterations.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115962865","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Evidence-Based Paediatric Oncology","authors":"L. Kremer, E. Loeffen, R. S. Phillips","doi":"10.1093/MED/9780198797210.003.0006","DOIUrl":"https://doi.org/10.1093/MED/9780198797210.003.0006","url":null,"abstract":"The practice of evidence-based medicine (EBM) is very important in delivering optimal patient care and the terms evidence-based medicine, or evidence-based practice, are used all around the world. This chapter discusses evidence-based paediatric oncology, including its history, an outline of what EBM is, EBM in paediatric oncology, steps in evidence-based paediatric oncology for a user of EBM, steps in guideline development as an implementer of EBM, common criticisms of EBM, and the future of EBM. The chapter gives an overview how EBM can be used in a non-exhaustive but still comprehensive way in daily practice of care for children with cancer, and which tools are available for paediatric oncologists. The majority of the chapter focuses on how to learn to become a skilled user of EBM.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"102 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124738062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bone Tumours","authors":"S. Bielack, M. Paulussen, L. Helman","doi":"10.1093/med/9780198797210.003.0026","DOIUrl":"https://doi.org/10.1093/med/9780198797210.003.0026","url":null,"abstract":"This chapter reviews the biology, diagnostics, multidisciplinary treatments, and prognosis of the most common bone sarcomas of childhood and adolescence—osteosarcoma and Ewing sarcoma. Both have their peak incidence among teenagers; both carry a high risk of metastatic spread. Local therapy alone is therefore rarely curative. Combining local therapies with intensive multidrug chemotherapy, usually employed both before and afterwards, can lead to long-term disease-free survival in approximately two thirds of affected young patients. Bone sarcomas should only be treated by experienced centres able to provide access to the full spectrum of required expert care. This includes access to essential diagnostic methods such as conventional radiography and magnetic resonance imaging (MRI) for evaluation of the primary, computed tomography (CT) of the chest to search for lung metastases, and radionuclide bone scan, positron emission tomography/computed tomography (PET/CT) and/or whole-body MRI to search for bone metastases. Local treatment of osteosarcoma is surgery whenever feasible. Limb-saving resections have largely replaced amputations. There is also a trend towards surgery in Ewing sarcoma, where radiotherapy has traditionally held a larger role. Histological response of the primary to preoperative chemotherapy is an important prognostic factor in both malignancies. Attempts to improve the prognosis of poor responders by postoperative treatment modifications have failed in osteosarcoma, but may work to some extent in Ewing sarcoma. Unfortunately, the many recent advances in the knowledge about their tumour biology have not yet resulted in better treatments for either of these two most common bone sarcomas of childhood and adolescence.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116471368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cancer in Adolescents and Young Adults","authors":"L. J. Jones, E. Saloustros, A. Ferrari, D. Stark","doi":"10.1093/med/9780198797210.003.0013","DOIUrl":"https://doi.org/10.1093/med/9780198797210.003.0013","url":null,"abstract":"Adolescents and young adults (AYAs) are a distinct group at the interface between children’s and adult’s cancer services. The distinctive pattern of cancers—biology, outcomes, psychosocial aspects, and late effects—mean they require specific clinical management and care. In the past, survival trends were stable or even deteriorating outcomes from AYA cancer. Then there was little research; treatment depended upon whether the managing clinician was accustomed to managing children or older adults, and was not tailored from specific knowledge and understanding. However, the last 20 years has seen greater interest, collaborative working; AYA-specific biology studies, clinical cohort and population-based studies; AYA-specific clinical services; and simultaneously improved survival. AYAs still pose significant challenges in care and treatment decisions, and in providing sufficient capacity and systems of care for all. Thus there remains much more to be done to further improve AYA cancer outcomes.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"5 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122290201","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Childhood Histiocytoses","authors":"M. Aricò, C. van den Bos, S. Weitzman","doi":"10.1093/med/9780198797210.003.0020","DOIUrl":"https://doi.org/10.1093/med/9780198797210.003.0020","url":null,"abstract":"This chapter summarizes the clinical spectrum of the histiocytic disorders—Langerhans cell histiocytosis (LCH), haemophagocytic lymphohistiocytosis (HLH), and some uncommon histiocytic disorders, including juvenile xanthogranuloma (JXG) and Rosai–Dorfman disease—as well as the current diagnostic and therapeutic approaches in these diseases. Multiple activating mutations in the RAS–RAF–MEK–ERK pathway have recently been described in LCH. Their role in the pathophysiology of the disorder and in targeted therapy is reviewed. This chapter explains the differences between primary and secondary HLH, and reviews the genetic abnormalities playing a role in both forms of HLH.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"36 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133710874","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Martha Grootenhuis, Momcilo Jankovic, Esther van den Bergh, Femke Aarsen
{"title":"Psychosocial Care","authors":"Martha Grootenhuis, Momcilo Jankovic, Esther van den Bergh, Femke Aarsen","doi":"10.32388/fneg7m","DOIUrl":"https://doi.org/10.32388/fneg7m","url":null,"abstract":"Children with cancer and their parents go through different phases of treatment which relate to different emotions and necessary psychosocial care. The stressors during the acute phase, the treatment phase, after treatment, long-term follow-up, or the palliative phase are described in this chapter. Consequences in terms of physical, social, emotional, and cognitive functioning are taken into account. Reactions of both children, parents, and siblings need to be considered, especially in relation to the age of the child. In terms of increased survival, it is important to acknowledge late effects and to teach survivors how to cope with a new normal.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"31 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-02-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130488812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Acute Myeloid Leukaemia","authors":"G. Kaspers, D. Reinhardt","doi":"10.1093/med/9780198746690.003.0515","DOIUrl":"https://doi.org/10.1093/med/9780198746690.003.0515","url":null,"abstract":"Acute myeloid leukaemia (AML) is the second most frequent type of leukaemia in children, and is cured nowadays in about 75% of patients in settings with essentially unlimited resources. This chapter discusses the aetiology and epidemiology of this interesting disease, its typical clinical presentation, diagnostics, and biology. The classifications, prognostic factors, and risk stratification (including minimal residual disease), and AML treatment (covering chemotherapy and allogeneic stem-cell transplantation) is presented. Several practical illustrations are included, and results of recent studies from the main cooperative study groups are summarized. Finally, side effects during treatment and late effects, as well as acute promyelocytic leukaemia (APL) and myeloid leukaemia of Down syndrome (ML-DS), are discussed. The chapter concludes with the actual challenges and perspectives.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"50 1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"116340486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Embryonal Tumours","authors":"M. Massimino, E. Bouffet, V. Ramaswamy","doi":"10.1201/9781315382715-40","DOIUrl":"https://doi.org/10.1201/9781315382715-40","url":null,"abstract":"Paediatric embryonal tumours are highly heterogeneous entities which account for 15–20% of all childhood tumours of the central nervous system (CNS). Although historically considered one entity, integrated genomic analysis has unveiled this is no longer the case, and in fact CNS-PNET (primitive neuroectodermal tumour) has been removed from the World Health Organization (WHO) classification of CNS tumours. Patients are risk-stratified based on residual disease after surgery, metastatic dissemination, and, with the medulloblastoma subgroup, specific molecular features. In patients with medulloblastoma, 60–70% of patients over three years old are classified as standard-risk cases, while high-risk patients include those with disseminated and/or residual disease, large-cell and/or anaplastic histotypes, and MYC gene amplification in some protocols. Atypical teratoid rhabdoid tumours (ATRTs) are risk-stratified in a similar manner; however, recently integrated genomics has revealed the presence of three distinct molecular variants which seem to have distinct clinical features and outcomes. Clinical trials already underway or currently being planned will (1) examine the feasibility of reducing the dose of craniospinal irradiation and the volume of posterior fossa radiotherapy (RT) for patients generally considered at low biological risk (i.e. those with the WNT subgroup of medulloblastomas; (2) ascertain whether intensifying chemotherapy or RT can improve outcomes in high-risk patients; and (3) seek therapeutic targets that will enable tailored therapies, especially for relapsing patients and those at higher biological risk.","PeriodicalId":375332,"journal":{"name":"Oxford Textbook of Cancer in Children","volume":"11 2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125819107","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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