PEDIATRIC ONCOLOGY Deborah Tomlinson Nancy E. Kline (Eds.) Pediatric Oncology Nursing Advanced Clinical Handbook Second Edition 123 Library of Congress Control Number 2009934491 ISBN 978-3-540-87983-1 e-ISBN 978-3-540-87984-8 Springer Heidelberg Dordrecht London New York ISSN 1613-5318 DOI: 10.1007/978-3-540-87984-8 © Springer-Verlag Berlin Heidelberg 2005, 2010 Deborah Tomlinson MN, RN This work is subject to copyright. All rights are reserved, Dip Cancer Nursing whether the whole or part of the material is concerned, specifi - Child Health Evaluative Sciences cally the rights oftranslation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any Hospital for Sick Children other way, and storage in data banks. Duplication of this publi- Child Health Evaluative cation or parts there of is permitted only under the provisions Toronto ON of the German Copyright Law of September 9, 1965, in its cur- Canada rent version, and permission for use must always be obtained from Springer-Verlag.Violations are liable for prosecution under the German Copyright Law. Nancy E.Kline PhD, RN, CPNP, FAAN, Springer is a part of Springer Science+Business Media Director, Research and Evidence-Based Practice springeronline.com Department of Nursing The use of general descriptive names, registered names, trade- Memorial Sloan-Kettering Cancer Center marks, etc. in this publication does not imply, even in the New York, NY absence of a specific statement, that such names are exempt USA from the relevant protective laws and regulations and there- fore free for general use. Cover design: eStudio Calamar, Figueres Berlin Product liability: The publishers cannot guarantee the accu- racy of any information about dosage and application con- tained in this book. In every individual case the user must check such information by consulting the relevant literature. Printed on acid-free paper 9 8 7 6 5 4 3 2 1 Springer.com V Dedication To all pediatric nurses. Your contribution to our world’s future is beyond words. To Chris and our children, Vivian, Sam, Suzanne and Angus – for giving me reason. Deborah Tomlinson To my parents, and my husband Michael. Thank you for your unwavering support throughout this endeavor. Nancy E. Kline VII Preface Pediatric Oncology Nursing: Advanced Clinical Hand- signs, diagnostic and laboratory procedures, treat- book is a joint effort between nurses in Canada, the ment, prognosis, and follow up care are included for United Kingdom, and the United States. This is the each of the disorders. second edition, and has been another wonderful Part III covers cancer treatment, including che- opportunity to bring together the expertise of hema- motherapy, radiation therapy, hematopoetic stem cell tology and oncology nurses from two continents. The transplantation, surgery, gene therapy, biotherapy, com- book is designed to be a comprehensive clinical hand- plimentary and alternative medicine, and clinical trials. book for nurses in advanced practice working with The principles and description of treatment, method pediatric hematology/oncology patients. Specifi c of treatment delivery, potential side effects, and special issues related to young children and adolescents with considerations for each type of treatment are discussed. cancer and hematologic disorders are discussed. Part IV focuses on the side effects of cancer Thirty-one contributors and two editors partici- treatment in relation to metabolic processes and pated in the writing of this text. Individuals in advanced gastrointestinal, hematologic, respiratory, renal, car- practice and academic roles – nurse practitioners, clin- diovascular, neurologic, musculoskeletal, integumen- ical nurse specialists, nutritionists, clinical instructors, tary, endocrine, and auditory systems. The incidence, lecturers, academicians, and educators – were involved. etiology, treatment, prevention, and prognosis are One of the most appealing features of this text is the included for each side effect reviewed. vast experience represented by our authors from differ- Part V includes the essential information regard- ent countries and different educational backgrounds. ing supportive and palliative care of pediatric cancer The book is divided into 5 sections: pediatric can- patients. Nutrition, hydration, pain, blood transfusion cers, hematologic disorders, treatment of childhood therapy, growth factors, and care of the dying child and cancer, side effects of treatment and disease, and sup- the family are covered. The principles of treatment for portive and palliative care. Many tables and illustra- these conditions, method of delivery, and special con- tions are included for quick reference in the clinical siderations for certain conditions are included. setting. Future perspectives and opportunities for The Editors of Pediatric Oncology Nursing: new treatment options and research are discussed. Advanced Clinical Handbook want to recognize, thank, Part I focuses on pediatric cancers – the leuke- and acknowledge everyone who participated in the mias, lymphomas, and solid tumors. The most com- development of this text. We are profoundly aware of mon pediatric tumors as well as some rare tumors the personal time and commitment that was devoted are discussed in regards to epidemiology, etiology, to make this an outstanding resource and we are grate- molecular genetics, symptoms and clinical signs, ful. It is our hope that nurses in advanced clinical prac- diagnostic and laboratory testing, staging and clas- tice will fi nd this publication useful, and that it will sifi cation, treatment, prognosis, and follow up care. enrich knowledge and improve care for children and Part II focuses on pediatric hematology. The ane- adolescents with cancer and hematologic disorders. mias, bleeding disorders, neutropenia, thrombo- cytopenia, and bleeding disorders are discussed in ON, Canada Deborah Tomlinson detail. Epidemiology, etiology, symptoms and clinical NY, USA Nancy E. Kline IX Contributors Linda D’Andrea MSN, RN, CS, CPON®, CCRN Angela M. Ethier PhD, RN, CNS, CPN, CT Memorial Sloan Kettering Cancer Center, Houston, TX, USA New York, NY, USA Lindsay Gainer MSN, RN Jane Belmore RSCN, RGN, Dip. Palliative Care Hematopoetic Stem Cell Transplant Unit, Schiehallion Day Care Unit, Yorkhill NHS Trust, Children’s Hospital Boston, Glasgow, Scotland, UK Boston, MA, USA Joan O’Brien Shea MSN, RN Sara Gonzalez MS, RD, LD Children’s Hospital Boston, Texas Children’s Hospital, Boston, MA, USA Houston, TX, USA Karyn Brundige MSN, RN, CPNP, ARNP Kristen Graham BSN, RN, CPON® Children’s Hospital and Regional Medical Center, Dana Farber Cancer Institute, Seattle, WA, USA Boston, MA, USA Rosalind Bryant MN, APRN-BC, PNP Ali Hall RSCN, RGN, BA, M.Phil., Baylor College of Medicine, Adv.Dip. Child Development , Pediatric Nurse Practitioner, Schiehallion Day Care Unit, Yorkhill NHS Trust, Texas Children’s Hospital, Glasgow, Scotland, UK Houston, TX, USA Joan O’Hanlon-Curry MS, RN, CPNP, CPON® Christine Chordas MSN, RN, CPNP Pediatric Hematology Oncology, Dana Farber Cancer Institute, The Children’s Hospital at Montefi ore, Boston, MA, USA Bronx, NY, USA Sandra Doyle MN, RN Eleanor Hendershot RN, BScN, MN Division of Haematology/Oncology, Division of Hematology/Oncology, Hospital for Sick Children, Hospital for Sick Children, Toronto, ON, Canada Toronto, ON, Canada Biljana Dzolganovski MMedSc, CCRP Jane Khorrami SRN Child Health Evaluative Services, Beatson West of Scotland Cancer Centre, Hospital for Sick Children, Gartnavel General Hospital, Toronto, ON, Canada Glasgow, Scotland X Contributors Nancy E. Kline PhD, RN, CPNP, FAAN Janice Post-White PhD, RN, FAAN Memorial Sloan Kettering Cancer Center, University of Minnesota School of Nursing, New York, NY, USA Minneapolis, MN, USA Elena Ladas MS, RD Fiona Reid RSCN, RN Columbia University, Morven House Raigmore Hospital, New York, NY, USA Inverness, UK Irene Loch RN, Dip Cancer Nursing Cheryl Rodgers MSN, RN, CPNP, CPON® Beatson West of Scotland Cancer Centre, Texas Children’s Cancer Center, Gartnavel General Hospital, Houston, TX, USA Glasgow, Scotland Carol Rossetto MSN, RN, CPNP, CPON ® Anne-Marie Maloney RN, BSc, MSc Memorial Sloan-Kettering Cancer Center, Division of Haematology/Oncology, New York, NY, USA Hospital for Sick Children, Toronto, ON, Canada Cara Simon PhD, RN, CPNP Texas Children’s Cancer Center, Martina Nathan RSCN, RGN, BSc, PGCE (M level), Houston, TX, USA ENB 240, ENB 998 , Cardiff University, Eastgate House, Cardiff, Deborah Tomlinson MN, RN, Wales, UK Dip Cancer Nursing , Child Health Evaluative Services, Colleen Nixon MSN, RN, CPON® Hospital for Sick Children, Children’s Hospital Boston , Toronto, ON, Canada Boston, MA, USA Julie Watson RN, MSN, CPNP Robbie Norville MSN, RN, CPON ® Complex Care Service, Paediatric Medicine, Texas Children’s Cancer Center, The Hospital for Sick Children, Houston, TX, USA Toronto, ON, Canada Margaret Parr RGN, RSCN, ENB240 Sue Zupanec RN, MN Children’s Services, Division of Hematology/Oncology, Queen’s Medical Centre, Hospital for Sick Children, Derby Road, Nottingham, UK Toronto, ON, Canada XI Contents PART I 2 Lymphoma 1 Leukemia Sue Zupanec Sue Zupanec • Deborah Tomlinson 2.1 Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . 33 1.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . 2 2.2 Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . 33 1.2 Acute Lymphoblastic Leukemia . . . . . . . . . . 3 2.2.1 Epidemiology . . . . . . . . . . . . . . . . . . 34 1.2.1 Epidemiology . . . . . . . . . . . . . . . . . . 3 2.2.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 34 1.2.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 4 2.2.3 Molecular Genetics. . . . . . . . . . . . . . . 34 1.2.3 Molecular Genetics. . . . . . . . . . . . . . . 6 2.2.4 Symptoms and Clinical Signs . . . . . . . . 34 1.2.4 Symptoms and Clinical Signs . . . . . . . . 7 2.2.5 Diagnostics . . . . . . . . . . . . . . . . . . . 36 1.2.5 Diagnostics . . . . . . . . . . . . . . . . . . . 8 2.2.6 Staging and Classifi cation . . . . . . . . . . 38 1.2.6 Staging and Classifi cation . . . . . . . . . . 9 2.2.7 Treatment . . . . . . . . . . . . . . . . . . . . 39 1.2.7 Treatment . . . . . . . . . . . . . . . . . . . . 12 2.2.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 42 1.2.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 16 2.2.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 43 1.2.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 16 2.2.10 Relapsed/Refractory HL. . . . . . . . . . . . 43 1.2.10 Future Perspectives . . . . . . . . . . . . . . 17 2.2.11 Future Perspectives . . . . . . . . . . . . . . 44 1.2.11 Relapsed ALL . . . . . . . . . . . . . . . . . . 17 2.3 Non-Hodgkin Lymphomas. . . . . . . . . . . . . . 44 1.3 Acute Myeloid Leukemia . . . . . . . . . . . . . . . 19 2.3.1 Epidemiology . . . . . . . . . . . . . . . . . . 44 1.3.1 Epidemiology . . . . . . . . . . . . . . . . . . 19 2.3.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 45 1.3.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 19 2.3.3 Molecular Genetics. . . . . . . . . . . . . . . 45 1.3.3 Molecular Genetics. . . . . . . . . . . . . . . 20 2.3.4 Symptoms and Clinical Signs . . . . . . . . 46 1.3.4 Symptoms and Clinical Signs . . . . . . . . 20 2.3.5 Diagnostics . . . . . . . . . . . . . . . . . . . 48 1.3.5 Diagnostics . . . . . . . . . . . . . . . . . . . 21 2.3.6 Staging and Classifi cation . . . . . . . . . . 50 1.3.6 Staging and Classifi cation . . . . . . . . . . 21 2.3.7 Treatment . . . . . . . . . . . . . . . . . . . . 50 1.3.7 Treatment . . . . . . . . . . . . . . . . . . . . 22 2.3.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 54 1.3.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 24 2.3.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 54 1.3.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 24 2.3.10 Treatment of Relapsed/Refractory NHL. . 55 1.3.10 Future Perspectives . . . . . . . . . . . . . . 24 2.3.11 Future Perspectives . . . . . . . . . . . . . . 55 1.4 Chronic Myeloid Leukemia. . . . . . . . . . . . . . 25 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56 1.4.1 Epidemiology and Etiology . . . . . . . . . 25 1.4.2 Molecular Genetics. . . . . . . . . . . . . . . 25 1.4.3 Symptoms and Clinical Signs . . . . . . . . 25 1.4.4 Diagnostics . . . . . . . . . . . . . . . . . . . 26 3 Solid Tumor 1.4.5 Treatment . . . . . . . . . . . . . . . . . . . . 26 Eleanor Hendershot 1.4.6 Prognosis. . . . . . . . . . . . . . . . . . . . . 26 1.4.7 Future Perspectives . . . . . . . . . . . . . . 26 3.1 Ewing’s Sarcoma Family of Tumors . . . . . . . . 60 1.5 Juvenile Myelomonocytic Leukemia . . . . . . . 26 3.1.1 Epidemiology . . . . . . . . . . . . . . . . . . 60 1.6 Langerhans Cell Histiocytosis. . . . . . . . . . . . 27 3.1.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 60 1.6.1 Epidemiology and Etiology . . . . . . . . . 27 3.1.3 Molecular Genetics. . . . . . . . . . . . . . . 60 1.6.2 Diagnostics . . . . . . . . . . . . . . . . . . . 28 3.1.4 Symptoms and Clinical Signs . . . . . . . . 61 1.6.3 Symptoms and Clinical Signs . . . . . . . . 28 3.1.5 Diagnosis. . . . . . . . . . . . . . . . . . . . . 61 1.6.4 Treatment . . . . . . . . . . . . . . . . . . . . 29 3.1.6 Staging and Classifi cation . . . . . . . . . . 63 1.6.5 Prognosis. . . . . . . . . . . . . . . . . . . . . 29 3.1.7 Treatment . . . . . . . . . . . . . . . . . . . . 63 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29 3.1.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 64 XII Contents 3.1.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 65 3.6.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 100 3.1.10 Future Perspectives . . . . . . . . . . . . . . 65 3.6.10 Future Directions. . . . . . . . . . . . . . . . 100 3.2 Osteosarcoma . . . . . . . . . . . . . . . . . . . . . . 65 3.7 Rhabdomyosarcoma. . . . . . . . . . . . . . . . . . 100 3.2.1 Epidemiology . . . . . . . . . . . . . . . . . . 66 3.7.1 Epidemiology . . . . . . . . . . . . . . . . . . 100 3.2.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 66 3.7.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 101 3.2.3 Molecular Genetics. . . . . . . . . . . . . . . 66 3.7.3 Molecular Genetics. . . . . . . . . . . . . . . 101 3.2.4 Signs and Symptoms . . . . . . . . . . . . . 67 3.7.4 Symptoms and Clinical Signs . . . . . . . . 101 3.2.5 Diagnostics . . . . . . . . . . . . . . . . . . . 67 3.7.5 Diagnostics . . . . . . . . . . . . . . . . . . . 101 3.2.6 Staging and Classifi cation . . . . . . . . . . 68 3.7.6 Staging and Classifi cation . . . . . . . . . . 103 3.2.7 Treatment . . . . . . . . . . . . . . . . . . . . 68 3.7.7 Treatment . . . . . . . . . . . . . . . . . . . . 103 3.2.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 71 3.7.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 106 3.2.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 71 3.7.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 106 3.2.10 Future Perspectives . . . . . . . . . . . . . . 71 3.7.10 Future Perspectives . . . . . . . . . . . . . . 106 3.3 Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . 72 3.8 Non-Rhabdomyosarcomatous 3.3.1 Epidemiology . . . . . . . . . . . . . . . . . . 72 Soft-Tissue Sarcomas . . . . . . . . . . . . . . . . . 107 3.3.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 72 3.8.1 Alveolar Soft-Part Sarcoma. . . . . . . . . . 108 3.3.3 Molecular Genetics. . . . . . . . . . . . . . . 72 3.8.2 Desmoid Tumor (Aggressive 3.3.4 Symptoms and Clinical Signs . . . . . . . . 72 Fibromatosis) . . . . . . . . . . . . . . . . . . 108 3.3.5 Diagnostics . . . . . . . . . . . . . . . . . . . 73 3.8.3 Desmoplastic Small Round Cell Tumor . . 108 3.3.6 Staging and Classifi cation . . . . . . . . . . 74 3.8.4 Infantile Fibrosarcoma . . . . . . . . . . . . 109 3.3.7 Treatment . . . . . . . . . . . . . . . . . . . . 75 3.8.5 Infantile Hemangiopericytoma . . . . . . . 109 3.3.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 76 3.8.6 Infantile Myofi bromatosis . . . . . . . . . . 109 3.3.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 76 3.8.7 Leiomyosarcoma. . . . . . . . . . . . . . . . 109 3.3.10 Future Perspectives . . . . . . . . . . . . . . 77 3.8.8 Liposarcoma. . . . . . . . . . . . . . . . . . . 109 3.4 Neuroblastoma . . . . . . . . . . . . . . . . . . . . . 77 3.8.9 Malignant Peripheral Nerve 3.4.1 Epidemiology . . . . . . . . . . . . . . . . . . 77 Sheath Tumor . . . . . . . . . . . . . . . . . 110 3.4.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 77 3.8.10 Synovial Sarcoma . . . . . . . . . . . . . . . 110 3.4.3 Molecular Genetics. . . . . . . . . . . . . . . 77 3.9 Germ Cell Tumors . . . . . . . . . . . . . . . . . . . 110 3.4.4 Symptoms and Clinical Signs . . . . . . . . 79 3.9.1 Epidemiology . . . . . . . . . . . . . . . . . . 111 3.4.5 Diagnostics . . . . . . . . . . . . . . . . . . . 80 3.9.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 111 3.4.6 Staging and Classifi cation . . . . . . . . . . 81 3.9.3 Molecular Genetics. . . . . . . . . . . . . . . 112 3.4.7 Treatment . . . . . . . . . . . . . . . . . . . . 82 3.9.4 Symptoms and Clinical Signs . . . . . . . . 112 3.4.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 85 3.9.5 Diagnostics . . . . . . . . . . . . . . . . . . . 112 3.4.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 85 3.9.6 Staging and Classifi cation . . . . . . . . . . 113 3.4.10 Future Perspectives . . . . . . . . . . . . . . 86 3.9.7 Treatment . . . . . . . . . . . . . . . . . . . . 113 3.5 Renal Tumors. . . . . . . . . . . . . . . . . . . . . . . 86 3.9.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 115 3.5.1 Epidemiology . . . . . . . . . . . . . . . . . . 86 3.9.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 115 3.5.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 87 3.9.10 Future Perspectives . . . . . . . . . . . . . . 116 3.5.3 Molecular Genetics. . . . . . . . . . . . . . . 87 3.10 Rare Tumors . . . . . . . . . . . . . . . . . . . . . . . 116 3.5.4 Symptoms and Clinical Signs . . . . . . . . 87 3.10.1 Adrenocortical Carcinoma . . . . . . . . . . 116 3.5.5 Diagnostics . . . . . . . . . . . . . . . . . . . 88 3.10.2 Melanoma . . . . . . . . . . . . . . . . . . . . 117 3.5.6 Staging and Classifi cation . . . . . . . . . . 89 3.10.3 Nasopharyngeal Carcinoma . . . . . . . . . 118 3.5.7 Treatment . . . . . . . . . . . . . . . . . . . . 89 3.10.4 Pleuropulmonary Blastoma . . . . . . . . . 118 3.5.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 92 3.10.5 Thyroid Carcinoma. . . . . . . . . . . . . . . 119 3.5.9 Follow-Up . . . . . . . . . . . . . . . . . . . . 93 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119 3.5.10 Future Perspectives . . . . . . . . . . . . . . 93 3.6 Retinoblastoma . . . . . . . . . . . . . . . . . . . . . 93 3.6.1 Epidemiology . . . . . . . . . . . . . . . . . . 93 3.6.2 Etiology. . . . . . . . . . . . . . . . . . . . . . 94 4 Central Nervous System Tumors 3.6.3 Molecular Genetics. . . . . . . . . . . . . . . 94 Nancy E. Kline • Joan O’Hanlon-Curry 3.6.4 Signs and Symptoms . . . . . . . . . . . . . 94 3.6.5 Diagnostics . . . . . . . . . . . . . . . . . . . 95 4.1 Causes/Epidemiology . . . . . . . . . . . . . . . . . 129 3.6.6 Staging and Classifi cation . . . . . . . . . . 96 4.2 Distribution/Classifi cation . . . . . . . . . . . . . . 129 3.6.7 Treatment . . . . . . . . . . . . . . . . . . . . 96 4.3 Staging . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 3.6.8 Prognosis. . . . . . . . . . . . . . . . . . . . . 99 4.4 Molecular Genetics of Brain Tumors . . . . . . . 130