Pediatric Oncology: Surgical and Medical Aspects Devendra K Gupta, Robert Carachi
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1Pediatric Oncology (Surgical and Medical Aspects)
Under the Aegis of the Indian Association of Pediatric Surgeons2
3Pediatric Oncology (Surgical and Medical Aspects)
Editors Devendra K Gupta MBBS, MS, MCh, FICS, FAMS, FAMS (Hony), FRCSG (Hony), DSc (Honoris Causa) Professor and Head, Department of Pediatric Surgery All India Institute of Medical Sciences, Ansari Nagar, New Delhi, INDIA Robert Carachi MD, PhD, FRCS (Glasgow), FRCS (Eng) Professor and Head, Section of Surgical Paediatrics Royal Hospital for Sick Children Yorkhill, Glasgow, Scotland, UK
4Published by
Jitendar P Vij
Jaypee Brothers Medical Publishers (P) Ltd
B-3, EMCA House, 23/23B Ansari Road, Daryaganj
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Pediatric Oncology (Surgical and Medical Aspects)
© 2007, Devendra K Gupta, Robert Carachi
All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the editors and the publisher.
First Edition: 2007
9788180619663
Typeset at JPBMP typesetting unit
Printed at Ajanta Press
5CONTRIBUTORS 9FOREWORD
“Twenty years from now you will be disappointed by the things you did not do rather than by the things you did. Catch the trade winds. Explore. Dream. Discover.” – Mark Twain
This multiauthor text on pediatric oncology edited by Prof. Devendra K Gupta and Prof. Robert Carachi fulfills an important need in the assessment, management, and long-term follow-up of children with cancer. Modern oncology has evolved remarkably over the past two decades, so that we are now able to treat the unique pathology and disease processes associated with pediatric tumors. Clinicians increasingly must have a comprehensive background in the molecular basis of cancer, as it takes a leading role in determining diagnosis and treatment.
Since 1960, overall survival has increased from 20 to 70 percent for most pediatric cancers. This stunning progress has been made through strict adherence to investigational protocols conducted by single institutions and multi-institutional study groups. These multidisciplinary protocols have made multi-agent chemotherapy, newer and more effective radiation therapy, and improved surgical techniques the standard of care. These improvements, combined with progress in diagnostic imaging (MRI, PET scans) and supportive care (antibiotics, nutrition, blood products, rehabilitation, pain control), have made treatment planning for childhood cancer safe, innovative, and effective.
The logical conclusion of improved long-term survival has been a shift in philosophy. The primary aim of treatment is now to reduce morbidity while maintaining disease-free survival. Modifications aimed at achieving that goal include: (a) decreased chemotherapy and radiation therapy for localized disease, (b) use of preoperative therapy to decrease the size of bulky tumors, and (c) changes in the role of surgery in relation to other therapies. The rapid pace of growth in knowledge, plus greater understanding of the molecular basis of cellular processes, improved biotechnology, and refinement of treatment modalities, has advanced every phase of care.
The 21st century may hold great opportunities and challenges in the care of children with cancer. The practicing oncologist—whether his specialty is medicine, radiation, or surgery—must be able to command a formidable knowledge base and to interact with colleagues in a wide range of disciplines. This text, written by multiple investigators and innovative oncologists, should be of value to all students, faculty members, and physicians, regardless of their years in practice.
Although it is gratifying to see how far the practice of pediatric oncology has come, it is also daunting to recognize how far it has yet to go.
“Once you start studying medicine you will never get through.” – CH Mayo
Bhaskar N Rao md, facs
Member, Department of Surgery
Director, Surgical Training Program
International Outreach Program
322 N. Lauderdale St. Memphis, TN38105-2794, USA10
11FOREWORD
Significant improvements in survival have been achieved for children with malignant solid tumors during the past three decades. This no doubt has been influenced by the establishment of multidisciplinary cancer treatment programs following carefully designed protocols as part of cooperative cancer group studies. Most of the research and progress in childhood cancer has occurred in developed countries, mainly in the West where a health care infrastructure can support these endeavors. Unfortunately, 60 percent of the children with cancer in the world have limited access to effective cancer care and their survival is significantly less than children residing in developed countries. Cancer has emerged as a major cause of childhood mortality of Asia, South and Central America, northwest and Sub-Saharan Africa, and the Middle East. For example, it is estimated that in China alone, a country with 300 million children, 45,000 new pediatric cancer cases will occur annually. It is therefore appropriate and timely that a textbook concerning childhood cancer be forthcoming from this region of the world. Pediatric Oncology (Surgical and Medical Aspects) is edited by Professors Devendra K Gupta and Robert Carachi.
The content of the book is exhaustive and covers a wide spectrum of both pediatric medical and surgical oncologic topics. Most of the contributors are cancer experts from Asian countries including India, Japan, China and Korea. Some of the contributors from Europe have been active in the SIOP study groups and a sparse number from the US and Africa involved with COG studies and similar cancer protocols. The content focuses mainly on surgical oncologic care. The text is divided into seven sections: the scientific basis of pediatric surgical oncology, clinical pediatric surgical and medical oncology, subspecialty tumors, endocrine and other rare tumors, therapeutic strategies (including chemotherapy, radiation therapy and some novel treatments) and long-term outcomes. The latter area is of special importance since a considerable number of pediatric cancer survivors experience adverse long-term side-effects related to intensive treatment and quality of life is an important parameter.
In regard to solid tumors, the pediatric surgical oncologist still plays a vital role in the care of the cancer patient. The survival of children with most solid malignancies is enhanced by complete removal of the primary tumor. The text carefully covers the surgical management of each of the major childhood tumors and the multimodal neoadjuvant or adjuvant therapy that results in improved survival. The editors have also included useful appendices that provide details regarding tumor protocols and chemotherapy schedules.
The textbook, originating in Asia, should provide a unique perspective for pediatric surgical oncologic care to the readers in India and other parts of Asia. Hopefully, this will also stimulate an improved understanding of the surgical aspects of pediatric cancer and nurture the development of cancer programs in less developed areas of the world. I congratulate the editors for putting together a very contemporary and informative textbook that should be a useful addition to the surgeon's library.
Jay L Grosfeld md
Lafayette F Page Professor of Pediatric Surgery, Emeritus
Indiana University School of Medicine
Indianapolis, Indiana, USA12
13PREFACE
Dealing with young children suffering from the most dreaded disease of malignancy has never been a pleasant experience. It requires tremendous zeal of courage to deal with the parents and help them cope up with the reality to put a brave front forward to win the battle. This is followed by an array of investigations in the right direction and then the appropriate treatment as decided by the cancer board. There are various gray areas in between the diagnosis and cure.
The incidence of cancer worldwide for age matched controls for all sites varies between 220 and 320 per 100,000 populations. The highest incidence is in the South American continent followed by Australia, Europe, Africa, North America and Asia in the decreasing order. The term ‘childhood cancer’ describes cancer diagnosed before the age of 15. This includes 30 percent of the total cases of cancer. About half of these die each year.
The cancers found in children are completely different from those seen in adults and these include leukemia, lymphomas, brain tumors, nephroblastoma, neuroblastoma and others and forms one of the leading causes of mortality the world over. It is the third main cause of deaths in children, after infections and malnutrition in developing countries and second most common cause in developed countries after trauma.
The treatment for pediatric cancer as in adults is multispecialty, involving pediatric surgeons, oncologists, radiotherapist, radiologist, pathologist and nuclear medicine specialist. An expert in the field of pain relief and nutrition is also needed for the terminally ill patients with incurable diseases. Though, a steady progress has been made over the past 40 years resulting in substantial improvement in the comprehensive medical and surgical care resulting in better quality of life of children suffering with malignancy yet, the children from developing world present very late with advanced stage of the disease, suffer from poor nutrition, anemia and bulky tumors. The resources are limited. The workload is too much. Also the expertise and the facilities remain limited and available mostly to the urban population.
There are presently about 40 pediatric centers in India providing surgical care to the children with various types of pediatric cancers. The number is grossly inadequate keeping in mind the massive workload from 400 million children in India alone. Chemotherapy drugs, though available, are expensive and toxic to our children. The facility for radiotherapy is available only at a few places. The overall cancer treatment is very expensive and remains beyond the reach of the common man. Though, a support from the Government, NGOs and philanthropists is made available from time to time, yet it remains inadequate and insufficient.
The Department of Pediatric Surgery at All India Institute of Medical Sciences, New Delhi has been actively involved with the pediatric cancer patients, providing surgery, chemotherapy, radiotherapy and other support. Many academic activities including the Pediatric Cancer Awareness Day and an International Symposium on Pediatric Oncology have been organized from time to time with the objectives to create awareness among the general public and to share the scientific knowledge with the experts. This recent symposium held during the Golden Jubilee of AIIMS, laid the foundation to publish this book on Pediatric Malignancies to serve as a reference point for various institutions and the teaching community, especially those serving in the developing world. AIIMS has developed a telemedicine link with the Royal Hospital for Sick Children, Yolkhill, Glasgow. This has led to various discussions on difficult tumor cases between the two institutions.14
The book has been divided into seven sections for easy understanding and smooth flow of ideas from one section to the other. on Basic Sciences provides an insight into the epidemiology, genetics, pathology and recent developments in the diagnosis of the various tumors. on Clinical Basis deals with the major tumors dealt by pediatric surgeons in great depth and deals with all minute details of handling these cases. on Medical Oncology deals with the thee major malignancies in children as dealt by the experts in their field would be beneficial to medical oncologists. The and on Subspecialities and Rare Tumors make this book complete in itself on all types of malignancies in children including bone, brain and eye. and deal with Therapeutic Strategies and Long-term Results in Pediatric Surgical Oncology.
We warmly acknowledge the support received from the faculty and the staff of our departments, in particular Dr Shilpa Sharma, Senior Research Associate, who has been working very hard, almost single handedly, reviewing and scrutinizing the manuscripts in detail before sending these to print.
It was a pleasant experience to work with M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, who agreed to publish this book at a reasonable cost. It is hoped that it would be useful to the students, teachers and the experts dealing with various types of cancers in children, working under suboptimal facilities.
Devendra K Gupta
Robert Carachi