The Surgery of Childhood Tumors-3판

Beginning with the scientific basis of tumors, this book provides up-to-date information on epidemiology,
cytogenetics, and molecular biology, before examining current treatments for the full range of pediatric tumors.
Integration of surgery, neoadjuvant and adjuvant chemotherapy, and radiation therapy is a dominant theme. In addition,
chapters on supportive care, palliative care, and the role of parents?associations reflect the book's holistic
approach.

All chapters are written by world-renowned international authorities on pediatric cancer from major children's cancer
groups. Excellent full-color pictures and line drawings illustrate all aspects of managing childhood tumors, including
details of operative techniques neglected in many other texts. This comprehensive book, expanded and updated to
encompass the very latest developments and strategies, provides a contemporary approach for pediatric, general, and
urological surgeons dealing with childhood tumors.


Contents
Contributors
1: A Brief History of Pediatric Oncology
About SIOP
History
Societe Internationale D뭀ncologie Pediatrique- International Society of Paediatric Oncology
Constitution
The International Society of Paediatric Surgical Oncology (IPSO)
Historical Background
IPSO
Aims
Development
A Brief History of Modern Pediatric Oncology in the United States
Children뭩 Oncology Group (COG)
Selected References
2: Epidemiology of Childhood Tumours
Classification
Incidence
Aetiology
Survival
Mortality
References
3: Tumor Biology and Environmental Carcinogenesis
Introduction
Cell Fate
Stem Cells
Programmed Cell Death
Malignant Transformation
DNA Content
Chromosomal Translocations
Proto-oncogene Activation
Inactivation of Tumor Suppressor Genes
Genetic Variants
Epigenetics
Metastasis
Angiogenesis
Environmental Carcinogenesis
Epidemiology
Potential Causative Agents
Radiation
Chemical Agents
Viruses
Parental Occupation and Exposure to Noxious Agents
Iatrogenic Factors
Tumor Types
Neuroblastoma
Wilms Tumor
Liver Tumors
Summary
References
4: Screening for Genetic Susceptibility to Paediatric Tumours
Introduction
Recognising an Underlying Genetic Susceptibility
Family History
Adult Tumours in Childhood
Familial Adenomatous Polyposis
Genetic Testing
Juvenile Polyposis
Genetic Testing
Screening
Peutz Jeghers (PJS)
Screening
The Tumour Is Frequently due to a Germ Line Susceptibility (Table 4.2)
Familial Retinoblastoma
Screening
WAGR, Denys-Drash and Frasier Syndrome
Clinical Screening for Wilms Tumour
SMARCB1 and Rhabdoid Tumours
Screening for SMARCB1 Families
DICER1 Syndrome and Pleuropulmonary Blastoma
Multiple Endocrine Neoplasia (MEN) Type 2 and Medullary Thyroid Cancer (MTC)
Endocrine Tumours
MEN 2B
MEN2A
Genetic Testing in MEN2
Biochemical Screening of Patients with MTC or MEN 2 Syndromes
Von Hippel Lindau Disease (VHL)
Familial Paraganglioma
Parathyroid Tumours
Genetic Testing
Clinical Screening
Phaeochromocytoma
MEN1
Hyperparathyroidism and Jaw Tumours
Autosomal Dominant Hyperparathyroidism
Genetic Testing
Clinical Screening for Hyperparathyroidism
Adrenocortical Tumours (ACT)
Pituitary Tumours
Screening in Pituitary Families and MEN1
Biochemical Screening in MEN1
Li Fraumeni
Screening for Li Fraumeni
The Carney Complex
McCune-Albright ?Polyostotic Fibrodysplasia
Genetic Testing
Differentiated Thyroid Cancer (DTC)
Tumours Associated with Abnormal Growth or Developmental Anomalies
Growth Disorder (Table 4.3)
Overgrowth and Macrocephaly
Beckwith-Wiedemann
Hemi Hypertrophy
Simpson Golabi Behmel
Perlman and 2q37.1 Deletions
Recommended Clinical Screening (Table 4.4)
Soto Syndrome and Weaver Syndrome
Cowden Syndrome and PTEN
Gorlin Syndrome, SUFU Mutations and Medulloblastoma
Regular Dermatolological Surveillance from BCCs is Mandatory in Gorlin
Abnormalities of Ras Signalling and Paediatric Tumours
Costello Syndrome
Neurofibromatosis Type 1
Growth Failure (Table 4.5)
DNA repair disorders
Base excision repair
MYH Polyposis
Nucleotide Excision Repair (NER)
Xeroderma Pigmentosa
Double Strand Break Repair
Fanconi뭩 Anaemia (FA)
Lynch Syndrome, Turcot and Mismatch Repair
Clinical Screening
Disorders Associated with RecQ Helicase Deficiencies
Mosaic Variegate Aneuploidy
Chromosomal Disorders (Table 4.6)
Genetic Testing (Table 4.7)
Summary
References
5: Genetic Counseling for Childhood Tumors and Inherited Cancer-쵵redisposing Syndromes
General Principles of Cancer Genetics
Genetic Counseling Aspects of Childhood Cancer
General Principles of Genetic Counseling
Genetic Counseling for Specific Tumor Types
Ependymoma
Ewing뭩 Sarcoma
Gliomas
Gonadoblastoma
Hepatoblastoma
Lymphoma
Medulloblastoma and Primitive Neuroectodermal Tumor (PNET)
Nephroblastoma (Wilms?Tumor)
Neuroblastoma
Osteogenic Sarcoma
Retinoblastoma
Rhabdomyosarcoma
Teratoma
Inherited Cancer-Predisposing Syndromes
PTEN Hamartoma Tumor Syndrome (Including Bannayan뻉iley뻉uvalcaba and Cowden Syndromes, OMIM 15
Beckwith뻎iedemann Syndrome (EMG Syndrome, OMIM 130650)
Blue Rubber Bleb Nevus Syndrome (Bean Syndrome, OMIM 112200)
Costello Syndrome
Denys뺻rash Syndrome (Wilms?Tumor and Pseudohermaphroditism, OMIM 194080)
Familial Adenomatous Polyposis Coli (FAP, OMIM 175100)
Gorlin뭩 Syndrome (Nevoid Basal Cell Carcinoma Syndrome, OMIM 109400)
Li뺽raumeni Syndrome (OMIM 151623)
McCune뺸lbright Syndrome (Polyostotic Fibrous Dysplasia, OMIM 174800)
Maffucci뭩 Syndrome (Osteochondromatosis, Multiple Enchondromatosis, Ollier Disease, OMIM 166000)
Multiple Exostoses (Diaphyseal Aclasis, Multiple Osteochondromatosis, OMIM 133700, 133701, 600209)
MYH-Associated Polyposis (OMIM 608456)
Neurofibromatosis Type 1 (OMIM 162200)
Neurofibromatosis Type 2 (OMIM 101000)
Perlman뭩 Syndrome (Renal Hamartomas, Nephroblastomatosis and Fetal Gigantism, OMIM 267000)
Rothmund뻋homson Syndrome (Poikiloderma Atrophicans and Cataract, OMIM 268400)
SOTOS Syndrome (Cerebral Gigantism, OMIM 117550)
Tuberous Sclerosis (OMIM 191100, 191092)
WAGR Syndrome (Wilms?Tumor, Aniridia, Genitourinary Anomalies, Mental Retardation Syndrome, OMIM
References
Websites
6: Tumour Markers
Introduction
Germ Cell Tumour Markers
Alpha-Fetoprotein
Human Chorionic Gonadotrophin
Clinical Applications of AFP and ?hCG
Other Markers for Germ Cell Tumours
Hepatic Tumour Markers
Alpha-Fetoprotein and Human Chorionic Gonadotrophin
Other Markers for Hepatic Tumours
Tumour Markers for Neuroblastoma and Related Tumours
Catecholamines and Metabolites
Phaeochromocytoma and Paraganglioma
Carcinoid Tumour
Other Tumour Markers for Neuroblastoma
Genetic and Biological Markers for Neuroblastoma
Screening for Neuroblastoma with Urinary VMA and HVA
Treatment of Neuroblastomas Detected on Screening
Effects of Neuroblastoma Mass Screening
Tumour Markers for Wilms Tumour
Tumor Markers for Other Tumors
Tumour Markers ?The Future
References
7: Tumor Imaging
General Principles
Plain Radiography
Ultrasound
Computerized Tomography
Magnetic Resonance Imaging
Nuclear Isotopes
Positron Emission Tomography (PET)
Neck Masses
Abdominal Masses
Thoracic Masses
Mediastinal and Chest Wall Tumors
Lymphoma
Teratoma
Neuroblastoma
Primitive Neuroectodermal Tumor (PNET)
Osteogenic Sarcoma and Ewing뭩 Tumors
Musculoskeletal Masses
Renal Tumors
Differential Diagnosis of Pediatric Renal Masses
Nephroblastoma (Wilms?Tumor)
Nephroblastomatosis
Mesoblastic Nephroma
Others
Neuroblastoma
Adrenocortical Tumors
Hemangioma
Abdominal B Cell Lymphoma
Rhabdomyosarcoma
Liver Tumors
Hepatoblastoma
Hepatocellular Carcinoma
Pelvic Tumors
Rhabdomyosarcoma
Teratoma
Second Malignant Neoplasms
References
8: Tumor Pathology: General Principles
Introduction
The Diagnostic Specimen
The Request Form
Cytology
The Diagnostic Needle Core Biopsy
Incisional Biopsy
Excisional Biopsy
Surgical Resection
Specimen Handling in the Pathology Department
Ancillary Studies
Fixation and Processing
The Preparation of Histological Material
The Diagnostic Process
Immunohistochemistry
Electron Microscopy
Cytogenetics
Molecular Genetics
The Prognostic Process
Standard Histological Criteria
Cytogenetics and Molecular Genetics
Additional Techniques
Flow Cytometry
Indices of Cell Proliferation in Tumors
p53
Future Perspectives
References
9: Chemotherapy and Novel Cancer Targeted Therapies
Introduction
Staging
Chemotherapy
The Cell Cycle and Tumour Growth Kinetics
Combination Chemotherapy
Adjuvant Chemotherapy
Dose Intensity
Management of Side Effects of Chemotherapy
Acute Complications
Tumour Lysis Syndrome
Bone Marrow Suppression
Infection
Nausea and Vomiting
Malnutrition/Mucositis
Late Effects
New Drugs for Children and Adolescents with Cancer
Targeted Therapy
Other Immunomodulators
Cancer Vaccination and T-cell Therapy
Future Challenges
References
10: Radiotherapy
Introduction
What Is Radiotherapy
Radiobiology
Type of Radiation
Total Dose
Dose Per Fraction
Interval Between Fractions
Overall Time
Typical Pediatric Treatment Schedules
Challenges of Pediatric Radiotherapy
Lowest Effective Total Dose
Smallest Possible Treatment Volume
Low Dose Rate or Dose Per Fraction
An Appropriate Overall Time
Procedures
Decision to Irradiate
Booking
Information Giving and Preparation
Anesthetic
Immobilization
Planning
Consent
Treatment
Palliative Treatment
Surgical Procedures to Reduce Toxicity of Radiotherapy
Timing of Radiotherapy with Respect to Surgery
Adverse Effects and Management
Acute Effects
Late Effects
Other Techniques
Brachytherapy
Stereotactic Radiosurgery (SRS)
Stereotactic Radiotherapy
Proton Beam Irradiation
Intensity Modulated Radiotherapy (IMRT)
Therapeutic Radioisotopes
References
11: Perinatal Tumors
Introduction
Overview
Epidemiology and Clinical Presentation
Oncogenesis and Genetic Risk Factors
Diagnostic Investigations
Therapeutic Interventions
Surgical Management
Radiotherapy
Chemotherapy
Teratomas
Sacrococcygeal Teratoma
Clinical Presentation and Diagnosis
Management Approaches
Operative Treatment
Fetal Surgery
Early Delivery
Postnatal Intervention
Adjuvant Chemotherapy
Long-Term Outcomes
Head and Neck Teratomas
Intracranial Teratomas
Cervical Teratomas
Retroperitoneal Teratomas
Mediastinal Teratomas
Neuroblastoma
Clinical Presentation
Diagnostic Evaluation
Antenatal
Postnatal
Stage 4S Disease
Treatment and Prognosis
Stages 1 and 2
Stages 3 and 4
Stage 4S
Soft Tissue Sarcomas
Rhabdomyosarcoma
Non-rhabdomyosarcoma Soft Tissue Sarcomas
Infantile Fibrosarcoma
Renal Tumors
Congenital Mesoblastic Nephroma
Wilms Tumor
Liver Tumors
Infantile Hepatic Hemangiomas
Mesenchymal Hamartomas
Hepatoblastoma
Retinoblastoma
References
12: Renal Tumors
History
Wilms Tumor Incidence and Etiology
Genetic Origins
Pathologic Precursors: Nephrogenic Rests, Nephroblastomatosis, and Multicystic Dysplastic Kidneys
Pathology of Renal Tumors
Clinical Presentation
Treatment
Chemotherapy
Preoperative Chemotherapy
Complications of Chemotherapy
Surgery
Surgical Details
Preoperative Therapy
Bilateral Wilms Tumor
Intravascular Extension
Surgery Alone for Select Favorable Wilms Tumors-Very Low Risk Wilms Tumors (VLRWT)
Neonatal Wilms Tumor
Extra-renal Wilms Tumor
Renal Cell Carcinoma
Mesoblastic Nephroma
Cystic Nephroma
References
13: Nephron-Sparing Surgery
Introduction
Current Indications for Nephron-Sparing Surgery
Evaluation and Operative Considerations
Influence of Surgical Approach on Renal Function of Children with Unilateral Renal Tumor: A Pe
Conclusions
References
14: Neuroblastoma and Other Adrenal Tumors
Adrenal Tumors
Embryology
Neuroblastoma
History
Incidence
Risk Factors
Associated Syndromes and Heredity
Clinical Presentation
Diagnostic Workup
Histopathology
Histology
Biologic Features
Staging
INRG Classification
Risk Groups
Low Risk
Definition
Treatment
Intermediate Risk
Definition
Treatment
High Risk
Definition
Treatment
Special Circumstances
Recurrent Neuroblastoma
Experimental Treatments
Other Adrenal Tumors
Pheochromocytoma
Adrenocortical Carcinoma
Aldosteronoma
Adrenal Adenoma
Adrenal Calcification
References
15: Gastrointestinal Tumors in Children
Introduction
Lymphoma
Gastrointestinal Stromal Tumor
Leiomyosarcoma/Leiomyoma
Colonic Carcinomas
Neuroendocrine/Carcinoid Tumor
Gastrointestinal Teratoma
Inflammatory Myofibroblastic Tumor
Polyps/Polyposis Syndromes
Familial Adenomatous Polyposis
Juvenile Polyps/Polyposis
Peutz-Jeghers Polyposis
References
16: Liver Tumors in Children
Historical Context
Differential Diagnosis
Malignant Tumors
Hepatoblastoma (HB)
Epidemiology, Biology, Genetics
Pathology
Imaging, Staging, PRETEXT, Risk Group Stratification
Radiographic Imaging
PRETEXT Group and PRETEXT Annotation Factors
RISK GROUP Stratification
Chemotherapy
Chemotherapy Toxicity
Surgery
Surgical Guidelines COG
Surgical Guidelines SIOPEL
Technique and Timing of Surgical Resection
Biopsy
Operative Technique
Surgical Treatment Options for Preoperative Tumor Rupture
Surgical Complications
Liver Transplant for HB
Multifocal PRETEXT IV
Solitary PRETEXT IV
PRETEXT III +P, +V
Transplant in Patients with Pulmonary Metastatic Tumor at Diagnosis
Rescue Transplant for Relapse or Persistent Tumor
Pediatric Liver Unresectable Tumor Observatory (PLUTO)
Surgery for Local Relapse in the Liver
Hepatic Arterial Chemo-embolization (HACE) and Trans-arterial Chemoembolization (TACE)
Hepatocellular Carcinoma
Epidemiology, Biology, Genetics
Pathology
Fibrolamellar Hepatocellular Carcinoma (FL-HCC)
Transitional Liver Cell Tumor (TLCT)
PRETEXT and Staging HCC
Chemotherapy
Antiangiogenesis, Sorafanib
Surgery
Liver Transplant for HCC in Children
TACE/HACE with Doxorubicin Beads or Yttrium Radioactive Beads
Pre-operative Portal Venous Embolization
Percutaneous Ablative Therapies
Rhabdoid Tumor
Hepatic Sarcomas
Biliary Rhabdomyosarcoma
Undifferentiated Embryonal Sarcoma of the Liver
Angiosarcoma
Metastatic and Other Malignant Tumors Involving the Liver
Metastatic Liver Tumors
Hepatic Involvement in Hematologic Malignancies
Hemophagocytic Lymphohistiocytosis (HLH)
Langerhans?Cell Histiocytosis (LCH)
Acute Megakaryoblastic Leukemia (AMKL)
Hepatic Veno-occlusive Disease (VOD)
Liver Tumors as Secondary Malignancies
Benign Tumors
Benign Epithelial Tumors
Focal Nodular Hyperplasia
Macroregenerative Nodules
Hepatic (Hepatocellular) Adenoma
Mesenchymal Hamartoma
Hepatobiliary Cystadenoma
Benign Vascular Tumors
Infantile Hepatic Hemangioma
Kaposiform Hemangioendothelioma
Epithelioid Hemangioendothelioma
Hepatic Teratoma
Inflammatory Myofibroblastic Tumor
References
17: Pancreatic Neoplasms in Children
Introduction
Classification and Staging
Individual Neoplasms
Pancreatoblastoma
Solid Pseudopapillary Neoplasm
Acinar Cell Neoplasms
Invasive Ductal Adenocarcinoma
Pancreatic Intraepithelial Neoplasia
Intraductal Pancreatic Neoplasms
Mucinous Cystic Neoplasms (MCN)
Serous Neoplasms
Mesenchymal Neoplasms
Pancreatic Lymphoma
Pancreatic Endocrine Neoplasms
Insulinoma
Gastrinoma
VIPoma
Glucagonoma
Congenital Hyperinsulinism
References
18: Genito-Urinary Tumours
Bladder-Prostate Tumours
Clinical and Radiological Evaluation
Diagnosis: Primary Surgery/Biopsy
Pathology and Initial Chemotherapy
Delayed Local Treatment
Tumours of the Female Genital Tract
Rhabdomyosarcoma of the Female Genital Tract
Malignant Germ Cell Tumours (MGC) of the Vagina
Clear Cell Adenocarcinoma of the Vagina
Scrotal Tumours
References
19: Malignant Germ Cell Tumors
Introduction
Embryology and Classification
Genetics and Risk Factors
Tumor Markers
Extragonadal Germ Cell Tumors
Sacrococcygeal Tumors
Abdominal/Retroperitoneal
Growing Teratoma
Mediastinal
Genital
Cervicofacial Teratoma
Testes
Ovary
Conclusion
References
20: Soft Tissue Sarcoma
Introduction
Nonrhabdomyosarcoma Soft Tissue Sarcomas
Epidemiology
Clinical Presentation
Diagnosis
Prognostic Factors
Staging
Treatment
Surgery
Lymph Node Dissection
Resection of Metastatic Disease
NRSTS Pediatric Histologies
Alveolar Soft Part Sarcoma
Desmoplastic Small Round Cell Tumor
Infantile Fibrosarcoma
Fibrohistiocytic Tumors
Leiomyosarcoma
Liposarcoma
Malignant Peripheral Nerve Sheath Tumor (MPNST)
Synovial Sarcoma
Rhabdomyosarcoma
Epidemiology
Pathology
Molecular Biology
Clinical Presentation
Diagnosis
Pretreatment Staging, Clinical Grouping, and Risk Group Classification
Treatment
Surgical Treatment
Primary Resection
Lymph Node Evaluation
Second Look Operation
Surgical Treatment of Recurrent and Metastatic Disease
Chemotherapy
Radiotherapy
Management by Site
Head and Neck
Genitourinary Sites
Bladder and Prostate
Paratesticular
Vulva, Vagina, and Uterus
Extremity
Other Sites
Trunk
Biliary Tract
Retroperitoneum and Pelvis
Perineum and Perianal
Outcome and Future Research
References
21: Lymphoma
Childhood Lymphoma
Hodgkin뭩 Disease
Histology/Tumor Markers
Diagnostic Evaluation and Staging System
Clinical Presentation/Surgical Issues
Treatment
Prognosis and Long-Term Effects
Non-Hodgkin뭩 Lymphoma
Histology/Tumor Markers
Mature B-cell Lymphomas
Lymphoblastic Lymphomas
Anaplastic Large Cell Lymphomas
Follicular
MALT
Diagnostic Evaluation/Staging System
Clinical Presentation and Surgical Issues
Mediastinum
Abdomen
Testis
Treatment
Treatment of Relapsed NHL
References
22: Melanoma and Other Skin Cancers
Melanoma
Epidemiology and Predisposing Conditions
Genetics and Pathology
Common
Rare
Clinical Presentation
Staging
Imaging
Surgery
Non-surgical Treatment
Non-melanoma Skin Cancers
Epidemiology
Pre-disposing Conditions and Lesions
Gorlin Syndrome/Naevoid Basal Cell Carcinoma Syndrome
Epidermodysplasia Verruciformis
Xeroderma Pigmentosum
Albinism
Ferguson-Smith Disease/Multiple Self-Healing Squamous Epithelioma
Sebaceous Naevus
Immunosuppression
Basal Cell Carcinoma
Pathology
Clinical Presentation
Staging
Imaging
Surgical Management
Mohs?Surgery
Non-surgical Treatment
Squamous Cell Carcinoma
Pathology
Clinical Presentation
Staging
Imaging
Surgical Management
Non-surgical Treatment
Dermatofibrosarcoma Protruberans
Epidemiology
Pathology
Clinical Presentation
Staging
Imaging
Surgical Management
Non-surgical Treatment
Cutaneous Lymphomas
Cutaneous Angiosarcoma
Teratomas
Juvenile Xanthogranuloma
References
23: Bone Tumors; Limb Salvage
Introduction
Presentation
Radiographic Investigations
Biopsy
Fine Needle Aspiration (FNA)
Needle Biopsy
Incisional Biopsy
Excisional Biopsy
Staging Notations
Treatment of Common Primary Bone Tumors
Benign Bone-Forming Tumors
Osteoma
Osteoid Osteoma
Osteoblastoma
Malignant Bone-Forming Tumors
Osteosarcoma
Parosteal Osteosarcoma
Periosteal Osteosarcoma
Benign Cartilage-Forming Tumors
Osteochondroma
Chondroblastoma
Chrondromyxoid Fibroma
Malignant Cartilage-Forming Tumors
Chondrosarcoma
Dedifferentiated Chondrosarcoma
Mesenchymal Chondrosarcoma
Clear Cell Chondrosarcoma
Benign Tumors of Histiocytic or Fibrohistiocytic Origin
Giant Cell Tumor
Non-ossifying Fibroma/Benign Fibrous Histiocytoma
Malignant Tumors of Histiocytic or Fibrohistiocytic Origin
Malignant Fibrous Histiocytoma
Bone Tumors of Vascular Origin
Hemangioendothelioma
Hemangiopericytoma
Angiosarcoma
Adamantinoma
Ewing뭩 Sarcoma
Simple Bone Cyst (Unicameral Bone Cyst)
Aneurysmal Bone Cyst
Miscellaneous Tumors of Soft Tissue and Bone
Myositis Ossificans
Surgical Management
Embolization
Curettage Alone
Curettage and Bone Graft
Excision Alone
Osteoarticular Fibular Transplantation
Bone Transportation
Surgery for Large Osteoarticular Defects
Overall Survival
Early Complications
Allograft Techniques
Endoprosthetic Techniques
Case 1
Case 2
Case 3
Case 4
Amputation and Disarticulation
Management of Local Recurrence
Rehabilitation
References
24: Head and Neck Tumors
Cervical Adenopathy
Hodgkin뭩 Disease
Pathology and Genetics
Clinical Presentation
Diagnostic Evaluation
Staging
Management
Non-Hodgkin뭩 Lymphoma
Pathology and Genetics
Clinical Presentation
Diagnostic Evaluation
Staging
Treatment
Thyroid Tumors
Pathology and Genetics
Clinical Presentation
Diagnostic Evaluation
Staging
Management
Postoperative Management
Neuroblastoma
Pathology and Genetics
Clinical Presentation
Diagnosis
Staging
Treatment
Rhabdomyosarcoma
Pathology and Genetics
Clinical Presentation
Diagnostic Evaluation
Staging
Treatment
Other Soft Tissue Sarcomas
Fibrosarcoma
Malignant Peripheral Nerve Sheath Tumor
Synovial Sarcoma
Hemangiopericytoma
Malignant Fibrous Histiocytoma
Alveolar Soft Part Sarcoma
Parathyroid Tumors
Germ Cell Tumors
Salivary Gland Tumors
Nasopharyngeal Carcinoma
References
25: Brain Tumors, Intraocular and Orbital Tumors
Phakomatoses
Neurofibromatoses
Tuberous Sclerosis
Von Hippel-Lindau Disease
Posterior Fossa Tumors
밅erebellar?Tumors
Cerebellar Astrocytomas
Medulloblastoma
Prognostic Factors
Ependymoma
Brain Stem Tumors
Diffuse Pontine Glioma
Focal Tumors
Exophytic Tumors
Cervicomedullary Tumor
Supratentorial Tumors
Cerebral Hemispheric Tumors
Astrocytomas
Ependymoma
Oligodendrogliomas and Oligoastrocytomas
Ganglioglioma
Primitive Neuroectodermal Tumors (PNET)
Meningiomas
Cerebral Metastases
Midline Tumors
Germ Cell Tumors
Optic Nerve/Chiasm and Hypothalamic Gliomas
Craniopharyngiomas
Pineal Region Tumors
Intraventricular Tumors
Choroid Plexus Tumors
Colloid Cysts
Tumors of the Skull
Dermoid and Epidermoid Tumors of the Skull
Fibrous Dysplasia
Langerhans?Cell Histiocytosis (Histiocytosis X)
Hemangioma
Osteoma
Aneurysmal Bone Cyst
Chordomas
Malignant Primary and Secondary Tumors
Spinal Cord Tumors
Extramedullary Spinal Tumors
Epidermoid and Dermoid Tumors
Teratoma
Meningioma
Nerve Sheath Tumors
Hemangioblastoma
Metastatic Disease
Intramedullary Tumors
Intraocular Tumors
Retinoblastoma
Inheritance Patterns
Clinical Presentation
Genetic Testing
Differential Diagnosis
Treatment
Treatment Principles
Supportive Treatment
Early Diagnosis- The Role of Imaging and Tissue Sampling
Screening for Retinoblastoma
Prognosis
Recurrence
Risk of Second Cancers and the Role of Long Term Surveillance
Research Directions
Medulloepithelioma
Malignant Melanoma of the Iris and Choroid
Juvenile Xanthogranuloma (JXG)
Intraocular Vascular Tumors
Choroidal Osteoma
Myogenic and Neurogenic Tumors
Orbital Tumors
Diagnosis
Primary Benign Orbital Tumors
Optic Nerve Glioma
Optic Nerve Meningioma
Primary Orbital Cysts
Malignant Orbital Tumors
Primary Benign Orbital Tumors
Other Causes of Proptosis in Childhood
References
26: Tumors of the Lung and Chest Wall
Lung Tumors: Benign
Inflammatory Myofibroblastic Tumor (Plasma Cell Granuloma)
Hamartoma
Lung Tumors: Malignant
Bronchial Adenoma
Bronchogenic Carcinoma
Pulmonary Blastoma
Rhabdomyosarcoma
Treatment of Metastatic Disease
Osteosarcoma
Soft Tissue Sarcoma
Wilms?Tumor
Tumors of the Chest Wall
Epidemiology
Presentation
Diagnostic Adjuncts
Diagnosis
Therapeutic Principles
Tumor Types
Chest Wall Tumors: Benign
Aneurismal Bone Cyst (ABCs)
Chondroma
Desmoid
Fibrous Dysplasia
Mesenchymal Hamartoma
Osteochondroma
Chest Wall Tumors: Malignant
Chondrosarcoma (CS)
Ewing뭩 Sarcoma Family/Primitive Neuroectodermal Tumors (EWS/PNET)
Fibrosarcoma (FS)
Osteosarcoma (OS)
Rhabdomyosarcoma (RMS)
References
27: Mediastinal Tumors
Introduction
Anatomy
Clinical Presentation and Diagnosis
Clinical Presentation
Investigation
Anterior Mediastinal Tumors
Lymphomas
Teratomas and Germ Cell Tumors
Teratomas
Malignant Germ Cell Tumors
Thymic Tumors
Lymphangiomas
Others
Middle Mediastinal Tumors
Posterior Mediastinal Tumors
Neurogenic Tumors
Others
Anesthetic Consideration for Patients with Mediastinal Tumors
Risk Assessment
Alternatives to General Anesthesia for Diagnosis
Management of General Anesthesia
Video Assisted Thoracoscopic Surgery (VATS)
Single Lung Ventilation
Positioning and Port Placement
Conclusion
References
28: Rare Tumors
Definition
IPSO Rare Tumor Registry
Benign (Pseudo)Tumors
Inflammatory Myofibroblastic Tumor
Xanthogranulomatous Pyelonephritis
Juvenile Xanthogranuloma
Langerhans?Cell Histiocytosis
Association of Malignancy and Langerhans?Cell Histocytosis
Fibromatosis
Vascular Tumors
Malignant Vascular Tumors
Hemangiopericytomas
Rare Tumors of the Head and Neck
Malignant Mesenchymoma
Brain Metastases in Children
Nasopharyngeal Carcinoma
Salivary Gland Carcinoma
Synovial Sarcoma of the Larynx in a Child
Rare Tumors of the Chest
Tumors of the Sternum
Mediastinum
Tracheal and Bronchial Tumors in Children
Primary Lung Tumors
Pleuropulmonary Blastoma
Cystic Adenomatoid Malformation of the Lung and Malignant Tumors
Tumors of the Diaphragm
Breast Masses
Epidemiology and Etiology
Diagnosis
Treatment
Rare Tumors of the Abdomen
Peritoneum, Omentum, and Mesentery
Peritoneal Mesothelioma
Desmoplastic Round Cell Tumors
Lipoblastoma and Liposarcoma
Gastrointestinal Tract
General
Carcinoma of the Esophagus
Smooth Muscle Tumors
Leiomyoma and Leiomyoblastoma
Leiomyosarcoma
GIST
Gastric Teratoma
Gastric Carcinoma
Intestinal Polyps and Polyposis Syndromes
Juvenile Polyps
Generalized Juvenile Polyposis
Peutz-Jeghers Syndrome
Familial Adenomatous Polyposis (FAP)
Colorectal Carcinoma
Carcinoid Tumors
Bile Ducts
Introduction
Rhabdomyosarcoma
Carcinoma
Pancreas
Introduction
Pancreatoblastoma
Solid Pseudopapillary Tumor; Papillary Cystic Tumor of the Pancreas; Frantz뭩 Tumor
Pancreatic Ductal Adenocarcinoma
Malignant Endocrine Pancreatic Tumors
Rare Tumors of the Genitourinary System
Renal Cell Carcinoma
Renal Cell Carcinoma in Association with Tuberous Sclerosis in Children
Transitional Cell Carcinoma of the Bladder
Cystic Partially Differentiated Nephroblastoma
B Cell Non-Hodgkin뭩 Lymphoma as a Primary Renal Tumor
Prostatic Non-Hodgkin뭩 Lymphoma
Testicular Tumors Before Puberty
Ectomesenchymoma
Malignant Melanoma in Children
References
29: Reconstructive Surgery
Introduction
Principles of Patient Care
Reconstructive Flaps
Workhorse Flaps
Latissimus Dorsi
Rectus Abdominis
Radial Forearm Flap
Anterolateral Thigh Flap
Fibula Flap
Gracilis Flap
Gastrocnemius Flaps
Other Flaps for Tumour Surgery
Skin Grafting
Tissue Expansion
Synthetic Materials
Dermal Regeneration Templates
Synthetic Materials for Chest Wall and Abdominal Reconstruction
Chest Wall Reconstruction
Skeletal Reconstruction
Soft Tissue Reconstruction
Long-Term Complications
Reconstructive Urology
Introduction
Before Reconstruction
Options for Reconstruction
Tumours Confined to the Detrusor
Ileocystoplasty
Trigone
Urethral Sphincter and Bladder Neck
Clean Intermittent Catherization
Long Term Complications of Urological Reconstruction
Surgical Complications
Renal Function
Metabolic Consequences
Tumour Formation
Vaginal Reconstruction
References
30: Reconstructive Surgery for Bone Tumors
Reconstructive Surgery for Malignant Bone Tumor
Computer Assisted Navigation in Sarcoma Surgery
Advances in Biological Limb Reconstruction Following Resection of Malignant Tumours in Children
Types of Biological Reconstruction
Extracorporeal Irradiation and Reimplantation
Alternatives to Limb Sparing Surgery
Amputations in Children ?Surgical Issues
Rotationplasty
References
31: Surgical Complications of Childhood Tumors
Rare Surgical Presentations of Cancer in Childhood
Soft Tissue Complications
Cellulitis
Superficial Abscesses
Radiation Necrosis
Necrotizing Fasciitis
Compartment Syndrome
Thoracic Complications
Superior Vena Caval Syndrome
Superior Mediastinal Syndrome
Pneumomediastinum, Pneumopericardium, and Surgical Emphysema
Cardiac Tamponade
Pleurodesis
Fungal Lung Lesions
Complications of Long Lines
Complications of Catheter Removal
Gastrointestinal Complications
Acute Abdominal Pain
Overview
The Role of Diagnostic Imaging
The Role of Endoscopy
Assessment of Acute Abdominal Pain in the Child with Malignant Disease
Metabolic Causes of the Acute Abdomen
Specific Causes of Abdominal Pain
Intestinal Obstruction
Radiation Enteritis
Treatment of Obstruction in the Terminally Ill Child
Neutropenic Enterocolitis
Pancreatitis
Cholecystitis
Ruptured Tumor
Gastrointestinal Bleeding
The Role of Diagnostic Imaging in Acute Gastrointestinal Bleeding
The Role of Endoscopy in Acute Gastrointestinal Bleeding
Algorithm for Management of Gastrointestinal Bleeding
Esophagitis in the Immunosuppressed Patient
Infectious Colitis
Perianal Lesions
Genitourinary Tract Complications
Hemorrhagic Cystitis
Incidence
Clinical Features
Diagnosis
Prevention
Treatment
Outcome
Other Conditions Affecting the Genitourinary Tract
Intestinal Graft-Versus-Host Disease
Clinical Features
Investigations
Other Problems in Bone Marrow Transplant Patients
Treatment
References
32: Organ Dysfunction, Second Malignant Neoplasms, and Survival
Introduction
Organ Dysfunction
Neurocognitive Effects
Pulmonary
Cardiovascular
Gastrointestinal
Renal
Auditory Dysfunction
Endocrine Abnormalities
Gonadal Function
Pregnancy Related Outcomes
Second Neoplasms
Breast Cancer
Thyroid Cancer
Sarcomas
Bone Cancer
Gastrointestinal and Genitourinary Cancers
Leukemia
Life Expectancy for Childhood Cancer Survivors
Conclusions
References
33: Management of Childhood Solid Tumours in Third World Countries
Health Economics
Patterns of Disease
Medical Manpower
Access to Health Care
The Internet
Co-Morbidity
Other Resource Constraints
What all this means
Summary
References
34: Supportive Care of Children with Cancer
Introduction
Clinical Trials
Multidisciplinary Team Working
Care at Home
Education
Siblings
Treatment
Chemotherapy
Radiotherapy
Surgery
Cell and Gene Therapies
Haematopoietic Stem Cell Transplantation
Side-Effects of Treatment
Nausea and Vomiting
Neutropenia
Biochemical Derangements
Anaemia
Thrombocytopenia
Mucositis
Anorexia and Malnutrition
Breaking Bad News
What to Tell the Child
The Impact of a Cancer Diagnosis
Adolescents
Transition to Palliative Care
At Home
Child뭩 Questions
Hospice Care
Symptom Control
Pain
Constipation
Nausea and Vomiting
Bleeding
Seizures
Cough
Anorexia
Bladder Retention
밓ust in Case?Boxes
End of Life Care
Spiritual Needs of Children and Families
Support for Staff
Parents Preparing for the Child뭩 Death
After the Child Dies
Siblings
Grandparents
The Professional
Conclusion
References
35: Long-Term Effects of Childhood Cancer Therapy on Growth and Fertility
Long-Term Effects of Childhood Cancer Therapy on Growth and Fertility
Introduction
Endocrine Late Effects
Direct Radiation Damage to the Hypothalamic Pituitary Axis (HPA)
Growth Hormone Deficiency
Abnormalities of Gonadotrophin Secretion
Fertility
The Effects of Chemotherapy
The Effects of Radiotherapy
Fertility Protection and Preservation
Conclusion
Second Tumors
Incidence and Associations
Risk Factors/Etiology
Summary
References
36: Minimally Invasive Surgery in the Diagnosis and Treatment of Childhood Cancer
Laparoscopy in Pediatric Oncology
Impact of Laparoscopy on Tumor Cell Behavior
Thoracoscopy in Pediatric Oncology
Literature Review
References
37: New Treatments and New Therapies
Introduction
Immunotherapy
Monoclonal Antibody Therapy
T Cell-Based Therapies
Nonspecific Immunotherapy
Adoptive Cell Transfer
Adoptive Cell Transfer Using Tumor-Infiltrating Lymphocytes (TIL)
Adoptive Cell Transfer Using Genetically Modified Autologous Peripheral Blood Lymphocytes (PBL)
Vaccine Therapy
Angiogenesis Inhibitors
Gene Therapy
Gene Transfer
Gene Selection
Vectors
Strategy
Oncolytic Virotherapy
Bone Marrow Protection
Antisense Strategies
Antisense Targets
Conclusion
References
38: Central Venous Access
Introduction
Indications for the Insertion of Central Lines
Administration of Chemotherapeutic Agents
Administration of Intravenous Alimentation
Resuscitation
Monitoring and Repeat Blood Sampling
Administration of Antibiotics
Repeated Transfusion of Blood and Blood Products
Hemodialysis
Methods of Venous Access
General Principles Applying to Gaining Intravenous Access: 밫he 5A뭩?
Peripheral Venous Access
Peripherally Sited Central Venous Access
Central Venous Access
Catheterization of the Subclavian Vein
Technique
Catheterization of the Femoral Vein
Catheterization of the Jugular Veins
Totally Implantable Devices
Complications of CVLS and Their Treatment
Summary
References
39: Pain Management
Introduction
Management of Pain in the Child with Advanced Malignancy
Medical Treatment
When the Drugs Don뭪 Work
Other Symptoms
The Future
References
40: Paediatric Palliative Care
Introduction
Service Development and Epidemiology
Models of Disease Trajectory
Symptoms at the End of Life
Barriers to Care
Communication
Guidelines on Talking about Death and Dying
Conclusion
References
41: Medical Law as Applied to Paediatric Oncology Surgery
Introduction
Capacity to Provide Consent
Children under the Age of 16 Who Lack Capacity
Children under 16 Who Can Demonstrate Their Capacity
Young People of 16 & 17 Years of Age
Disclosure
The Standard of the Particular Patient
The Professional Standard
The Reasonable Patient Standard
The Numeric Threshold of Risk
References
42: Training in Paediatric Oncology and Tumour Boards
Training
Training in Paediatric Oncology
Paediatric Oncology National Training Number (NTN) Grid
Paediatric Oncology Higher Specialist Training Record (HST Record)
Grid Trainee Assessment
Paediatric Oncology Trainee Group (POTG)
Shared Care
Tumour Boards
References
43: International Confederation of Childhood Cancer Parent Organizations Parent Organisations: Part
CCI Activities to Help Parent Organisations Worldwide to Achieve Their Mission
Information and Sharing
Public Awareness
Development
Advocacy
Local Parent Organisations and Their Activities
National Parent Organisations and Their Activities
Index