Oncology of Infancy and Childhood: Expert Consult - Online and Print

To Address The Growing Complexities Of Childhood Cancer, Nathan And Oski₂s Hematology And Oncology Of Infancy And Childhood Has Now Been Separated Into Two Distinct Volumes. With This Volume Devoted Strictly To Pediatric Oncology, And Another To Pediatric Hematology, You Will Be On The Cutting Edge Of These Two Fields. This Exciting New, Full-color Reference Provides You With The Most Comprehensive, Authoritative, Up-to-date Information For Diagnosing And Treating Children With Cancer. It Brings Together The Pathophysiology Of Disease With Detailed Clinical Guidance On Diagnosis And Management For The Full Range Of Childhood Cancers, Including Aspects Important In Optimal Supportive Care. Written By The Leading Names In Pediatric Oncology, This Resource Is An Essential Tool For All Who Care For Pediatric Cancer Patients. And, As An Expert Consult Title, This Brand-new Resource Comes With Access To The Complete Contents Online, Fully Searchable. Offers Comprehensive Coverage Of All Pediatric Cancers, Including Less Common Tumors, Making This The Most Complete Guide To Pediatric Cancer. Covers Emerging Research Developments In Cancer Biology And Therapeutics, Both Globally And In Specific Pediatric Tumors. Includes A Section On Supportive Care In Pediatric Oncology, Written By Authors Who Represent The Critical Subdisciplines Involved In This Important Aspect Of Pediatric Oncology. Uses Many Boxes, Graphs, And Tables To Highlight Complex Clinical Diagnostic And Management Guidelines. Presents A Full-color Design That Includes Clear Illustrative Examples Of The Relevant Pathology And Clinical Issues, For Quick Access To The Answers You Need. Provides Access To The Complete Contents Online, Fully Searchable, Enabling You To Consult It Rapidly From Any Computer With An Internet Connection. Incorporates The Codified Who Classification For All Lymphomas And Leukemias. Stuart H. Orkin ... [et Al.]. An Expert Consult Title--cover. Includes Bibliographical References And Index. Mode Of Access: World Wide Web. Cover......Page 1 ISBN: 9781416034315......Page 2 Copyright Page......Page 3 Contributors......Page 6 Preface......Page 13 I - Biology of Cancer......Page 14 Epidemiology of Leukemia in Childhood......Page 16 Acute Lymphoblastic Leukemia......Page 17 Time Trends......Page 19 Leukemia and Cancer in the Families of Children with Leukemia......Page 20 Genetic Polymorphisms......Page 21 Residence in Areas with High Population Mixing......Page 22 Immunizations......Page 23 Seasonal Variations in Childhood Leukemia Onset......Page 24 Radiation Exposure......Page 25 Hydrocarbons and Solvents......Page 26 Maternal Diet and Vitamin Supplement Use During Pregnancy......Page 27 Maternal Age and Birth Order......Page 28 Congenital Anomalies......Page 29 REFERENCES......Page 30 Angiogenesis......Page 40 Molecular Players That Regulate Angiogenesis......Page 41 Inhibitors of Angiogenesis......Page 42 Hematologic Malignancies Are Angiogenic......Page 43 Myelodysplastic Syndrome......Page 44 Targeting the Vascular Endothelial Growth Factor Pathway......Page 45 Antiangiogenic Chemotherapy......Page 46 Platelets and Angiogenesis......Page 47 Capacity of Platelets to Regulate Angiogenesis......Page 48 REFERENCES......Page 49 Molecular Basis of Human Malignancy......Page 54 Cell-Intrinsic Events......Page 55 Telomeres and Telomerase in Human Cancer......Page 56 p53.......Page 57 Transformation......Page 58 Ras......Page 59 Myc......Page 60 Genetic Instability......Page 61 Cancer Cell Invasion and Metastasis......Page 62 REFERENCES......Page 63 Targeted Approaches to Drug Development......Page 70 Inhibitors of Microtubule Formation......Page 71 Toxicity Acceptable with Curative Therapy......Page 72 What is Targeted Therapy?......Page 73 Advantages of Targeted Therapy......Page 74 Synthetic Lethal or Empirical Track......Page 75 Target Expression......Page 76 Loss-of-Function Genetic Alterations......Page 77 Gain-of-Function Experiments......Page 79 Drugs......Page 80 Targeted Therapy: Lessons Learned from Adult Oncology......Page 83 Imatinib in Chronic Myelogenous Leukemia......Page 84 Beyond Chronic Myelogenous Leukemia and Gastrointestinal Stromal Tumors......Page 86 Epithelial Growth Factor Receptor Inhibition for Non–Small Cell Lung Cancer......Page 87 Rituximab Therapy for Non-Hodgkin’s Lymphoma......Page 88 Bevacizumab and Colorectal Cancer......Page 89 Heat Shock Protein 90 Inhibitors......Page 90 Histone Deacetylase Inhibitors......Page 91 Rarity of Pediatric Cancer......Page 92 Fms-Like Tyrosine Kinase 3 and Acute Leukemias......Page 93 Sonic Hedgehog Signaling and Medulloblastoma......Page 94 Transcription Factors and Pediatric Cancer......Page 96 Antidisialoganglioside and Neuroblastoma......Page 98 Insulin-like Growth Factor Receptor Signaling and Pediatric Solid Tumors......Page 99 REFERENCES......Page 100 Cytogenetic and Molecular Pathology of Pediatric Cancer......Page 112 Need for Sufficient Viable Tumor......Page 113 Unpredictable Tumor Cell Growth in Culture......Page 118 Complex Karyotype......Page 119 Technical Limitations in Detecting Aberrations......Page 120 Highly Combinatorial Modifications of Fluorescence in Situ Hybridization.......Page 121 Maximizing Data Yield from Individual Fluorescence in Situ Hybridization Probes for Rearrangements.......Page 123 General Applications of Polymerase Chain Reaction–Based Methods......Page 124 Specificity of Reverse Transcriptase Polymerase Chain Reaction.......Page 125 Quantitative Polymerase Chain Reaction......Page 126 GENOMIC MECHANISMS IN PEDIATRIC TUMORS......Page 127 Causes and Predisposing Factors for Chromosomal Translocations......Page 129 Chromosomal Rearrangements Involving Transcription Factor Genes......Page 131 Chromosomal Rearrangements Involving Protein Tyrosine Kinase Genes......Page 132 BIOLOGIC BASIS FOR SPECIFICITY OF BALANCED CHROMOSOMAL REARRANGEMENTS: DIAGNOSTIC RELEVANCE......Page 134 Ewing’s Sarcoma......Page 135 Rhabdomyosarcoma......Page 136 Clear Cell Sarcoma: Malignant Melanoma of Soft Parts......Page 138 Desmoplastic Small Round Cell Tumor......Page 139 Infantile Fibrosarcoma......Page 140 Neuroblastoma......Page 141 Pediatric Renal Cell Carcinomas with Xp11 or 6p21 Translocations......Page 142 Mesoblastic Nephroma......Page 143 CNS Tumors......Page 144 Hodgkin’s Lymphoma......Page 145 Nodular Lymphocyte-Predominance Hodgkin’s Lymphoma......Page 146 Anaplastic Large Cell Lymphoma......Page 147 Acute Lymphoblastic Leukemia......Page 149 Intralocus IG and TCR Gene Rearrangements......Page 150 Interlocus IG and TCR Gene Rearrangements and Translocations......Page 151 MLL Translocations.......Page 152 FLT3 Mutations.......Page 153 Acute Myeloid Leukemia......Page 154 t(15;17).......Page 156 t(6;9).......Page 157 Therapy-Related Acute Myeloid Leukemias and Myelodysplastic Syndromes......Page 158 Juvenile Myelomonocytic Leukemia......Page 159 Transient Abnormal Myelopoiesis and Acute Megakaryoblastic Leukemia of Down Syndrome......Page 160 RAS Mutations......Page 161 RELEVANCE OF GENETIC ABERRATIONS FOR TARGETED THERAPIES......Page 162 Imatinib Mesylate......Page 164 REFERENCES......Page 165 II - Pediatric Cancer Therapeutics......Page 185 Chemotherapy in the Pediatric Patient......Page 188 RATIONALE FOR CHEMOTHERAPY OPTIMIZATION......Page 189 Absorption Changes......Page 195 Hepatic Metabolism......Page 196 Choice of Starting Dose......Page 197 Coadministration of Other Chemotherapeutic Drugs......Page 198 Coadministration of Nonchemotherapeutic Drugs......Page 199 Coadministration of Complementary and Alternative Medicine......Page 200 Thiopurine S-Methyltransferase......Page 202 CYP2B6......Page 203 CYP2D6......Page 204 CYP3A4 and CYP3A5......Page 205 ABCB1......Page 206 Conventional Method: Body Surface Area–Based Dosing......Page 209 Therapeutic drug monitoring......Page 210 Pharmacokinetic alterations......Page 211 Drug scheduling and administration sequencing......Page 212 REFERENCES......Page 213 Immunotherapy of Cancer......Page 222 Monoclonal Antibodies......Page 223 T Cells......Page 225 γδ T Cells......Page 226 Dendritic Cells......Page 227 TUMOR TARGET ANTIGENS......Page 228 Normal Proteins Expressed in Immune-Privileged Sites......Page 229 Rationale and Physiology......Page 230 Immunization to Tumor Antigens......Page 231 The Interferons......Page 233 Interleukin-2......Page 234 MONOCLONAL ANTIBODIES......Page 235 Naked Monoclonal Antibodies......Page 236 Immunotoxins......Page 237 Radioimmunoconjugates......Page 238 Adoptive T-Cell Therapy......Page 239 Adoptive T-Cell Therapy to Treat Pediatric Malignancies......Page 240 The Graft Versus Leukemia Reaction......Page 241 Nonmyeloablative Transplants for Leukemia and Lymphoma......Page 243 Donor Lymphocyte Infusions......Page 244 Allogeneic Natural Killer-Cell Infusions......Page 245 CHALLENGES AND FUTURE DIRECTIONS......Page 246 REFERENCES......Page 247 Pediatric Radiation Oncology......Page 254 THE PHYSICAL BASIS OF RADIATION THERAPY......Page 255 Clonogenic Survival Curves......Page 256 Radiation Interactions with Chemotherapeutic Agents......Page 257 Vascular Endothelial Growth Factor Receptor Inhibitors......Page 258 Combining Molecularly Targeted Therapies with Conventional Antineoplastic Treatments......Page 259 Modern Treatment Approaches......Page 260 Intensity-Modulated Radiation Therapy......Page 261 Carbon Ions......Page 262 Clinical Applications......Page 263 Sequelae of Treatment......Page 265 REFERENCES......Page 266 Pediatric Surgical Oncology......Page 270 ANESTHESIA IN A PATIENT WITH AN ANTERIOR MEDIASTINAL MASS......Page 271 The Five Principles......Page 273 Small Specimen Biopsies......Page 274 Fine Needle Aspiration and Core Needle Biopsy......Page 275 Large Specimen Biopsies......Page 277 Extremity Tumors......Page 278 Wilms’ Tumor......Page 279 Neuroblastoma......Page 280 Hepatoblastoma......Page 282 Osteosarcoma, Ewing’s Sarcoma......Page 283 Surgical Principles......Page 284 Endoprostheses......Page 286 Distal Femur and Proximal Tibia......Page 287 Rotationplasty......Page 289 Pelvis......Page 290 Proximal Humerus......Page 292 Outcomes......Page 293 MANAGEMENT OF PULMONARY METASTASIS IN PEDIATRIC SOLID TUMORS......Page 294 The Four Principles......Page 295 Metastasis Localization......Page 296 Differentiated Thyroid Cancers......Page 297 Hepatoblastomas......Page 298 Sarcomas......Page 299 REFERENCES......Page 300 III - Hematologic Malignancy......Page 307 Acute Lymphoblastic Leukemia......Page 310 Immunophenotype......Page 311 B-precursor cell Acute Lymphoblastic Leukemia......Page 312 CLINICAL MANIFESTATIONS......Page 314 Bone Marrow......Page 315 Bone and Joint Manifestations......Page 316 Aplastic Anemia, Myelodysplasia, and Myeloproliferative Disorders......Page 317 Immunophenotype......Page 318 Minimal Residual Disease.......Page 319 Risk-Adapted Therapy......Page 320 Phases of Therapy......Page 321 Central Nervous System Treatment......Page 322 Continuation Therapy......Page 323 Philadelphia Chromosome–Positive Acute Lymphoblastic Leukemia......Page 324 Metabolic Complications......Page 325 Acute Neurologic Toxicity......Page 326 Bony Morbidity......Page 327 RELAPSED ACUTE LYMPHOBLASTIC LEUKEMIA......Page 328 Unrelated and Autologous Stem Cell Transplantation......Page 329 REFERENCES......Page 330 Myeloid Leukemia, Myelodysplasia, and Myeloproliferative Disease in Children......Page 344 Epidemiology and Etiology......Page 345 Clonal Origin of Myeloid Leukemia Cells......Page 346 Transformation of Stem Cells with Self-Renewal Capacity......Page 347 Molecular Genetics......Page 348 Class I Mutations......Page 349 Translocations Involving the Retinoic Acid Receptor-α.......Page 351 Type 2 Mutations......Page 353 Morphology and Cytochemistry......Page 354 M0: Acute Myeloblastic Leukemia with Minimal Differentiation......Page 359 M5: Acute Monocytic Leukemia......Page 361 World Health Organization Classification......Page 362 Remission Induction......Page 363 Current U.S. Trials......Page 364 Supportive Therapy at Diagnosis and During Therapy......Page 365 Bleeding Complications......Page 366 Chemotherapy Complications......Page 367 Demographic Risk Factors......Page 368 Prognosis in Relapsed or Refractory Disease......Page 369 Antibody Therapy with Gemtuzumab Ozogamicin......Page 370 Apoptosis inhibitors......Page 371 Arsenic trioxide......Page 372 Hematopoietic Stem Cell Transplantation......Page 373 Pathobiology......Page 374 Clinical Presentation......Page 375 Treatment......Page 376 Pathophysiology......Page 377 Clinical Presentation......Page 378 Pathobiology......Page 379 Chronic Myelogenous Leukemia Caused by the BCR-ABL Fusion Gene......Page 380 Clinical Presentation......Page 381 Tyrosine Kinase Inhibitors......Page 382 Hematopoietic Stem Cell Transplantation......Page 383 Polycythemia Vera......Page 384 Essential Thrombocythemia......Page 385 Agnogenic Myeloid Metaplasia with Myelofibrosis......Page 386 REFERENCES......Page 387 Infant Leukemias......Page 416 RISK FACTORS......Page 417 biology of Mixed Lineage Leukemia translocations......Page 418 Clinical and Biologic Features......Page 419 Prognostic Factors and Outcomes......Page 420 Toxicity and Central Nervous System–Directed Therapy......Page 421 Association with Down Syndrome......Page 422 Modulators of Glucocorticoid Resistance......Page 423 REFERENCES......Page 424 Malignant Lymphomas and Lymphadenopathies......Page 430 Histopathology and Classification......Page 431 Classic Hodgkin’s Lymphoma......Page 432 Nodular Lymphocyte-Predominant Hodgkin’s Lymphoma......Page 433 Epidemiology and Causative Factors......Page 434 Cellular Microenvironment: Cytokines and Chemokines......Page 437 Clinical Characteristics......Page 438 Clinical Evaluation and Staging Procedures......Page 441 Prognostic Factors—Stratification of Treatment......Page 444 Radiotherapy......Page 445 Combined-Modality Chemotherapy and Radiotherapy......Page 446 Relapsed Patients......Page 453 Late Effects......Page 455 Definition......Page 458 Immunophenotype......Page 459 Role of T-Cell Receptor Translocations.......Page 460 NOTCH1 Activation.......Page 462 HOX11(TLX1), HOX11L2(TLX3), and HOXA9 Homeobox Genes.......Page 463 Cell Cycle Regulators: p15INK4B, p14ARF, and p16INK4A Inactivation and Cyclin D2 Overexpression.......Page 464 Morphology.......Page 465 Role of Epstein-Barr Virus......Page 466 Centroblastic Variant.......Page 467 Anaplastic Large Cell Lymphoma......Page 468 Time Trends in Incidence Rates......Page 469 Familial Predisposition......Page 470 Infections......Page 471 Clinical Characteristics......Page 472 Diagnosis and Staging......Page 477 Staging Classification......Page 478 Clinical Evaluation and Staging Procedures......Page 479 Emergencies......Page 480 Prevention and Treatment of Acute Tumor Cell Lysis Syndrome......Page 481 Acute Renal Dysfunction and Uremia......Page 482 Chemotherapy......Page 483 Extracompartmental Therapy......Page 485 Chemotherapy......Page 486 Extracompartmental Therapy......Page 489 Chemotherapy......Page 491 Incomplete Tumor Regression, Second-Look Surgery, and Monitoring of Response......Page 492 Treatment of Children and Adolescents with Rare Non-Hodgkin’s Lymphoma Subtypes......Page 493 Treatment of Relapse......Page 494 Prognostic Factors......Page 495 Post–Solid Organ Transplantation PTLD Versus Post-HSCT PTLD......Page 496 Treatment......Page 497 LYMPHADENOPATHY......Page 498 Approach to the Patient......Page 499 MEDIASTINAL MASSES......Page 500 References......Page 501 IV - Solid Tumors......Page 519 Neuroblastoma......Page 522 Development of the Sympathetic Nervous System......Page 523 Hereditary predisposition......Page 525 MYCN Amplification......Page 526 Chromosome 17q Gain......Page 527 Neurotrophin Expression in Neuroblastoma......Page 528 Apoptosis Pathways......Page 529 Histopathology......Page 530 CLINICAL PRESENTATION......Page 532 Paraneoplastic Syndromes......Page 533 Risk Stratification......Page 534 Clinical Variables......Page 536 Treatment of Low-Risk Disease......Page 537 Treatment of Intermediate-Risk Disease......Page 538 Local Control......Page 539 Consolidation Therapy......Page 540 Treatment of Minimal Residual Disease......Page 541 Immunotherapy......Page 542 REFERENCES......Page 543 Pediatric Renal Tumors......Page 554 Staging......Page 555 Epithelial Patterns.......Page 556 Focal and Diffuse Anaplasia.......Page 557 Nephrogenic Rests and Nephroblastomatosis......Page 558 Classic Pattern.......Page 559 Clear Cell Sarcoma of the Kidney Versus Wilms’ Tumor.......Page 560 Differential Diagnosis......Page 561 Malignant Rhabdoid Tumor Versus Wilms’ Tumor.......Page 562 Xp11.2 Translocation Renal Cell Carcinomas......Page 563 Oncocytic Renal Carcinomas in Neuroblastoma Patients......Page 564 Epidemiology......Page 565 β-Catenin and WTX......Page 566 WT2—The 11p15 Locus......Page 567 Clinical Presentation and Diagnostic Evaluation......Page 568 Radiation Therapy......Page 569 Chemotherapy......Page 571 Anaplastic Wilms’ Tumor......Page 572 Late Effects of Wilms’ Tumor Treatment......Page 573 Treatment......Page 574 Clinical Presentation and Diagnostic Evaluation......Page 575 Molecular Biology and Genetics......Page 576 Treatment......Page 577 FUTURE DIRECTIONS......Page 578 REFERENCES......Page 579 Retinoblastoma......Page 588 CLINICAL PRESENTATION......Page 589 DIAGNOSIS......Page 590 Screening All Children......Page 591 Screening Children with Increased Risk for Retinoblastoma......Page 592 Routes of Extraocular Spread......Page 593 Histopathologic Features that Predict Extraocular Spread......Page 594 Relationship to Retinoblastoma......Page 595 Gene and Protein Structure......Page 596 Gene Mutations in RB1......Page 597 GENETIC DIAGNOSIS......Page 598 Management of Retinoblastoma......Page 599 Systemic Workup......Page 600 General Principles in Treating Intraocular Retinoblastoma......Page 601 Cryotherapy......Page 602 Radiotherapy for Retinoblastoma......Page 603 Chemotherapy for Retinoblastoma......Page 605 REFERENCES......Page 609 Tumors of the Brain and Spinal Cord......Page 614 EPIDEMIOLOGY......Page 615 Neural Tube......Page 617 Cerebellar Cortex......Page 618 Neurofibromatosis Type 1......Page 619 Gorlin’s Syndrome......Page 620 Lhermitte-Duclos Disease, Cowden’s Syndrome, and PTEN Mutation......Page 621 CONCEPTUAL ORGANIZATION OF PEDIATRIC BRAIN TUMORS......Page 622 Obstructive Hydrocephalus and Raised Intracranial Pressure......Page 624 Neuroimaging......Page 625 Neurosurgery......Page 628 Acute Management Issues......Page 629 Perioperative Issues......Page 631 Stereotactic Radiotherapy.......Page 632 Chemotherapy......Page 633 Blood-Brain Barrier......Page 634 Small-Molecule Inhibitors......Page 635 Gliomas......Page 636 Imaging and Histology.......Page 637 Management.......Page 638 Clinical Presentation.......Page 640 Management.......Page 641 Management.......Page 642 Management.......Page 643 Imaging and Histology.......Page 644 Pilomyxoid Astrocytomas......Page 645 Ganglioglioma and Glial-Neuronal Tumors......Page 646 Imaging and Histology.......Page 647 Subependymal Giant Cell Astrocytomas (SEGAs)......Page 648 Management.......Page 649 Lipoastrocytomas.......Page 650 Imaging and Histology.......Page 651 Management.......Page 652 Clinical Presentation.......Page 653 Management.......Page 654 Management.......Page 655 Clinical Presentation.......Page 656 Imaging and Histology.......Page 657 Prognosis.......Page 658 Management.......Page 659 Embryonal Tumors......Page 660 Clinical Presentation.......Page 661 Imaging and Histology.......Page 662 Management.......Page 664 Historical Perspective......Page 665 Radiation Therapy......Page 666 Prognosis......Page 667 Management.......Page 668 Management.......Page 669 Imaging and Histology.......Page 670 Ependymoblastomas......Page 671 Prognosis.......Page 672 Imaging and Histology.......Page 673 Imaging and Histology.......Page 674 Choroid Plexus Tumors......Page 675 Imaging and Histology.......Page 676 Clinical Presentation.......Page 677 Imaging and Histology.......Page 678 Management.......Page 679 Imaging and Histology.......Page 680 Prognosis.......Page 681 Management.......Page 682 Specific Management Issues in Infants and Young Children......Page 683 Infant Central Nervous System Primitive Neuroectodermal Tumors and Pineoblastoma......Page 684 Infant Glioma......Page 685 Imaging and Histology.......Page 686 Management.......Page 687 Hamartomas......Page 688 Astroblastomas......Page 689 Imaging and Histology.......Page 690 Imaging and Histology.......Page 691 Clinical Presentation.......Page 692 Management.......Page 693 Imaging and Histology.......Page 694 Late Effects of Radiation......Page 695 PALLIATIVE CARE......Page 698 REFERENCES......Page 699 Hepatoblastomas and Other Liver Tumors......Page 734 STEM CELL ORIGINS OF HEPATOBLASTOMA......Page 735 GENETIC PREDISPOSITION AND LIVER TUMORIGENESIS......Page 736 PREMATURITY AS A RISK FACTOR......Page 738 STAGING......Page 739 ACQUIRED GENETIC CHANGES IN HEPATOBLASTOMA......Page 740 TREATMENT: CHEMOTHERAPY......Page 741 SURGICAL ASPECTS OF LIVER TUMORS......Page 745 LESS COMMON MALIGNANT TUMORS OF THE LIVER......Page 746 BENIGN TUMORS OF THE LIVER......Page 747 REFERENCES......Page 748 Rhabdomyosarcoma......Page 756 Pathologic Classification of Rhabdomyosarcoma......Page 757 Li-Fraumeni Syndrome......Page 758 Costello Syndrome......Page 759 Comparative Genomic Hybridization Analyses of Copy Number Changes in Rhabdomyosarcoma......Page 760 Molecular Genetics of Chromosomal Translocations in Alveolar Rhabdomyosarcoma......Page 762 Gene Amplification in Rhabdomyosarcoma......Page 763 Oncogene and Tumor Suppressor Gene Mutations in Rhabdomyosarcoma......Page 764 Myogenic Pathways in the Tumorigenesis of Rhabdomyosarcoma......Page 765 Role of Insulin-like Growth Factors in Rhabdomyosarcoma......Page 766 Metastatic Pathways in Rhabdomyosarcoma......Page 767 Activation of the Shh Signaling Pathway......Page 769 Models in Other Species......Page 771 Gene Expression Profiling with Microarrays......Page 772 Detection of Minimal Disseminated Rhabdomyocarcoma......Page 773 Clinical Evaluation......Page 774 Histology......Page 775 Metastases at Diagnosis......Page 776 Cyclophosphamide......Page 777 Doxorubicin......Page 778 Irinotecan......Page 779 Treatment Strategies......Page 780 Surgery......Page 781 Radiation Therapy......Page 782 Infants and Toddlers......Page 783 Parameningeal Tumors......Page 784 Late Effects......Page 785 REFERENCES......Page 786 Nonrhabdomyosarcomas and Other Soft Tissue Tumors......Page 796 introduction......Page 797 Epidemiology......Page 798 Presentation and Evaluation......Page 800 Staging and Grading......Page 801 Treatment......Page 802 Chemotherapy......Page 803 ADIPOCYTIC TUMORS......Page 804 Infantile Myofibroma/Myofibromatosis and Hemangiopericytoma......Page 805 Specific Forms of Fibrosarcoma......Page 806 Low-Grade Fibromyxoid Sarcoma......Page 807 Myxofibrosarcoma......Page 808 Inflammatory Myofibroblastic Tumor......Page 809 Sporadic Leiomyosarcoma......Page 810 Giant Cell Angioblastoma......Page 813 Kaposi Sarcoma......Page 814 Mesenchymal Chondrosarcoma......Page 815 Synovial Sarcoma......Page 816 Clear Cell Sarcoma......Page 818 Desmoplastic Small Round Cell Tumor......Page 820 Angiomatoid Fibrous Histiocytoma......Page 821 Epithelioid Sarcoma......Page 822 Gastrointestinal Stromal Tumor......Page 823 Malignant Peripheral Nerve Sheath Tumors......Page 824 Undifferentiated Sarcoma/High-grade Undifferentiated Polymorphous Sarcoma......Page 825 REFERENCES......Page 826 Ewing’s Sarcoma......Page 842 EPIDEMIOLOGY......Page 843 Chromosomal Rearrangements in Ewing’s Sarcoma......Page 844 Wild-type EWS Protein......Page 845 EWS/FLI and Other TET/ETS Fusions in Ewing’s Sarcoma......Page 846 Ewing’s Sarcoma Cell of Origin......Page 848 EWS/FLI Target Genes......Page 849 Cooperative Pathways in Ewing’s Sarcoma Oncogenesis......Page 850 Clinical Presentation......Page 851 Imaging Features......Page 853 Pathologic Diagnosis......Page 854 PROGNOSTIC FACTORS......Page 855 Local Control......Page 857 Early Improvements with Vincristine, Actinomycin D, Doxorubicin, and Cyclophosphamide......Page 859 Addition of Ifosfamide and Ifosfamide plus Etoposide to VACA-Based Therapy......Page 861 Dose-Intensified VACA/IE Regimens......Page 862 Ewing’s Sarcoma of the Spine......Page 863 Role of Surgery and Radiation......Page 864 Role of High-Dose Therapy with Hematopoietic Stem Cell Rescue......Page 865 Systemic Chemotherapy......Page 866 LATE EFFECTS IN PATIENTS TREATED FOR EWING’S SARCOMA......Page 867 REFERENCES......Page 869 Osteosarcoma......Page 884 EPIDEMIOLOGY......Page 885 CAUSE......Page 886 Radiologic Evaluation......Page 888 Biopsy......Page 890 Pathology......Page 891 Staging......Page 894 Prognostic Factors......Page 895 Genetic Alterations......Page 896 Retinoblastoma......Page 897 p53......Page 899 MET......Page 900 Insulin-like Growth Factor......Page 901 Determinants of Metastasis......Page 902 Biologic Markers of Response to Chemotherapy......Page 903 Chemotherapy and Outcome......Page 904 Ifosfamide and Etoposide......Page 907 Neoadjuvant Chemotherapy......Page 908 Current Approach to Therapy......Page 909 Treatment of Initially Metastatic Osteosarcoma......Page 910 Treatment of Recurrent Osteosarcoma......Page 911 Complications of Therapy......Page 912 REFERENCES......Page 914 Pediatric Germ Cell Tumors......Page 924 Primordial Germ Cell Migration......Page 925 Testicular Germ Cell Tumors......Page 926 Histopathology......Page 928 Ovarian Teratoma......Page 929 Histopathology......Page 930 Seminoma......Page 931 Choriocarcinoma......Page 932 Incidence......Page 933 Possible Environmental Causes......Page 936 Cryptorchidism and Testicular Cancer......Page 937 Adolescent and Adult Malignant Germ Cell Tumors......Page 938 Testicular Germ Cell Tumors......Page 939 Sacrococcygeal Germ Cell Tumors......Page 940 Evaluation for Metastatic Disease......Page 941 Tumor Markers......Page 942 Surgical Approach to Sacrococcygeal Tumors......Page 944 Evaluation of Retroperitoneal Nodes in Pediatric Testicular Germ Cell Tumors.......Page 945 Prognostic Factors at Diagnosis......Page 946 Seminoma and Dysgerminoma......Page 947 Factors that Predict Relapse.......Page 948 History of Use of Chemotherapy In Men with Testicular Cancer.......Page 949 Treatment of Pediatric Germ Cell Tumors in the United States......Page 950 Treatment of Pediatric Germ Cell Tumors in the United Kingdom......Page 952 Dose of Bleomycin in Treatment of Pediatric Germ Cell Tumors......Page 953 Mature and Immature Teratomas......Page 954 Sacrococcygeal Tumors......Page 955 Men with Testicular Cancer......Page 956 Radiotherapy......Page 957 Prospects for Targeted Therapy of Germ Cell Tumors......Page 958 Risk of Cardiovascular Disease......Page 959 Gonadal Function.......Page 960 REFERENCES......Page 961 Histiocytoses......Page 976 Types of Dendritic Cells......Page 977 Incidence and Etiology......Page 978 Bone......Page 979 Skin......Page 980 Risk Sites......Page 981 Therapy of Single-System Disease......Page 982 Therapy of Multisystem Disease......Page 983 Refractory Langerhans Cell Histiocytosis......Page 984 Juvenile Xanthogranuloma......Page 985 Origins of Macrophages......Page 986 Nonsyndromic Familial Hemophagocytic Lymphohistiocytosis......Page 987 Hemophagocytic Lymphohistiocytosis Associated with Other Genetic Syndromes......Page 988 Macrophage Activation Syndrome......Page 989 Clinical Features......Page 990 Infection-Associated Hemophagocytic Lymphohistiocytosis.......Page 992 Hematopoietic Cell Transplantation......Page 993 REFERENCES......Page 994 Rare Tumors of Childhood......Page 1002 Presentation......Page 1003 Epidemiology......Page 1004 Clinical Presentation and Staging......Page 1005 BREAST TUMORS......Page 1006 Bronchial Adenomas......Page 1007 Bronchogenic Carcinoma......Page 1008 Other Pulmonary Tumors......Page 1009 Gastrointestinal Stromal Tumors in Adults......Page 1010 Pediatric Gastrointestinal Stromal Tumors......Page 1011 CARCINOID......Page 1012 COLORECTAL CARCINOMA......Page 1013 Treatment......Page 1014 OVARIAN TUMORS......Page 1016 TUMORS OF THE CERVIX AND VAGINA......Page 1017 MELANOMA......Page 1018 Other Factors......Page 1019 Treatment and Outcome......Page 1020 REFERENCES......Page 1021 V - Supportive Care......Page 1027 Diagnostic Imaging in the Evaluation of Childhood Cancer......Page 1030 Computed Tomography......Page 1031 Positron Emission Tomography......Page 1033 Staging Considerations......Page 1034 Oligodendroglioma......Page 1035 Central Neurocytoma......Page 1036 Infratentorial Tumors......Page 1037 Cerebellar Astrocytoma......Page 1038 Ependymoma......Page 1039 Metastatic Disease......Page 1040 Spinal Cord Tumors......Page 1044 Thyroid and Parathyroid Tumors......Page 1045 Mediastinal Masses and Tumors......Page 1046 Lymphoma......Page 1047 Thymoma......Page 1048 Tracheobronchial Tree Masses......Page 1049 Posterior Mediastinal Masses......Page 1051 Ewing’s Sarcoma......Page 1055 Pulmonary Metastases......Page 1057 Hepatoblastoma......Page 1058 Hepatocellular Carcinoma......Page 1059 Mesenchymal Hamartoma......Page 1061 Focal Nodular Hyperplasia......Page 1063 Spleen Tumors......Page 1064 Pancreatoblastoma......Page 1065 Pancreatic Sarcomas......Page 1066 Alimentary Tract Tumors......Page 1067 Wilms’ Tumor......Page 1069 Clear Cell Sarcoma......Page 1071 Renal Cell Carcinoma......Page 1072 Rare Renal Tumors......Page 1073 Rhabdomyosarcomas......Page 1075 Neuroblastoma......Page 1076 Ovarian Germ Cell Tumors......Page 1080 Testicular Germ Cell Tumors......Page 1081 Sacrococcygeal Germ Cell Tumors......Page 1082 MUSCULOSKELETAL NEOPLASMS......Page 1083 Osteosarcoma......Page 1084 Ewing’s Sarcoma......Page 1085 Other Bone Tumors......Page 1086 Synovial Sarcoma......Page 1088 Infantile (Desmoid-type) Fibromatosis......Page 1089 Acute Lymphoblastic Leukemia......Page 1090 Childhood Acute Myelogenous Leukemia......Page 1091 Lymphoma......Page 1092 Staging......Page 1094 Non-Hodgkin’s Lymphoma......Page 1095 Sites of Relapse......Page 1098 IMAGING TREATMENT COMPLICATIONS......Page 1099 NEW IMAGING TECHNIQUES......Page 1101 Magnetic Resonance Spectroscopy......Page 1102 Targeted Contrast Agents......Page 1103 REFERENCES......Page 1104 Infectious Diseases in Pediatric Cancer......Page 1112 Fungi......Page 1113 Physical Defense Barriers......Page 1114 Defects in Cell-Mediated Immunity......Page 1115 Fever and Neutropenia......Page 1116 Antibiotic Management of a Neutropenic Patient Who Becomes Febrile......Page 1117 Modification of Antibiotic Therapy During the Course of Granulocytopenia......Page 1118 Empirical Antifungal or Antiviral Therapy During Prolonged Granulocytopenia......Page 1119 Oropharyngeal Candidiasis (Thrush) and Candida Esophagitis......Page 1121 Chronic Disseminated (Hepatosplenic) Candidiasis......Page 1122 Infections Caused by Aspergillus Species......Page 1123 Infection by the Varicella-Zoster Virus......Page 1124 Other Viral Infections......Page 1125 REFERENCES......Page 1126 Oncologic Emergencies......Page 1134 Overview......Page 1135 Incidence......Page 1136 Clinical Manifestations and Treatment......Page 1137 Fluids and Alkalinization......Page 1138 Hyperuricemia......Page 1139 Definition......Page 1141 Clinical Presentation......Page 1142 Treatment......Page 1143 ANTERIOR MEDIASTINAL MASSES......Page 1144 Evaluation and Imaging......Page 1147 Chemotherapy......Page 1149 Clinical Studies......Page 1150 Vesicant Extravasation......Page 1152 REFERENCES......Page 1153 Nursing Care of Patients with Childhood Cancer......Page 1158 OVERVIEW OF THE PEDIATRIC ONCOLOGY NURSE’S ROLE......Page 1159 The Nurse’s Relationship with Patients, Families, and Other Providers......Page 1160 Patient- and Family-Center To Address The Growing Complexities Of Childhood Cancer, Nathan And Oski2s Hematology And Oncology Of Infancy And Childhood Has Now Been Separated Into Two Distinct Volumes. With This Volume Devoted Strictly To Pediatric Oncology, And Another To Pediatric Hematology, You Will Be On The Cutting Edge Of These Two Fields. This Exciting New, Full-color Reference Provides You With The Most Comprehensive, Authoritative, Up-to-date Information For Diagnosing And Treating Children With Cancer. It Brings Together The Pathophysiology Of Disease With Detailed Clinical Guidance On Diagnosis And Management For The Full Range Of Childhood Cancers, Including Aspects Important In Optimal Supportive Care. Written By The Leading Names In Pediatric Oncology, This Resource Is An Essential Tool For All Who Care For Pediatric Cancer Patients. And, As An Expert Consult Title, This Brand-new Resource Comes With Access To The Complete Contents Online, Fully Searchable. Offers Comprehensive Coverage Of All Pediatric Cancers, Including Less Common Tumors, Making This The Most Complete Guide To Pediatric Cancer. Covers Emerging Research Developments In Cancer Biology And Therapeutics, Both Globally And In Specific Pediatric Tumors. Includes A Section On Supportive Care In Pediatric Oncology, Written By Authors Who Represent The Critical Subdisciplines Involved In This Important Aspect Of Pediatric Oncology. Uses Many Boxes, Graphs, And Tables To Highlight Complex Clinical Diagnostic And Management Guidelines. Presents A Full-color Design That Includes Clear Illustrative Examples Of The Relevant Pathology And Clinical Issues, For Quick Access To The Answers You Need. Provides Access To The Complete Contents Online, Fully Searchable, Enabling You To Consult It Rapidly From Any Computer With An Internet Connection. Incorporates The Codified Who Classification For All Lymphomas And Leukemias. Stuart H. Orkin ... [et Al.]. An Expert Consult Title--cover. Includes Bibliographical References And Index. Mode Of Access: World Wide Web.