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Advances in Management of Pediatric Brain Tumors
Mohamed S. Abdelbaki, Paul B. Fisher, David D. Limbrick.
Advances in Management of Pediatric Brain Tumors, Volume 167 encapsulates the latest developments in the field for various types of pediatric brain tumors.By examining these survival gaps, the volume offers insights into strategies for improving outcomes and bridging the gap in care on a global scale.
Front Cover -- Advances in Cancer Research -- Copyright -- Contents -- Contributors -- Preface -- Chapter One: Pediatric-Type Diffuse Low Grade Glioma -- 1 Introduction -- 2 The molecular evolution of pediatric-type diffuse low-grade gliomas -- 3 An approach to integrated pDLGG diagnosis -- 4 Diffuse astrocytoma, MYB/MYBL1 altered -- 4.1 Epidemiology and clinical presentation -- 4.2 Imaging features -- 4.3 Histopathologic and molecular diagnosis -- 4.4 Treatment approach and outcomes -- 5 Angiocentric glioma -- 5.1 Epidemiology and clinical features -- 5.2 Imaging features -- 5.3 Histopathologic and molecular diagnosis -- 5.4 Treatment approach and outcomes -- 6 Polymorphous low-grade neuroepithelial tumor of the young -- 6.1 Epidemiology and clinical features -- 6.2 Imaging features -- 6.3 Histopathologic and molecular diagnosis -- 6.4 Treatment approach and outcomes -- 7 Diffuse pediatric low-grade glioma, MAPK-altered -- 7.1 Epidemiology and clinical features -- 7.2 Imaging features -- 7.3 Histopathologic and molecular diagnosis -- 7.4 Treatment approach and outcomes -- 7.5 Management of BRAFV600E mutated diffuse PLGG -- 7.6 Management of FGFR1-altered pDLGG -- 7.7 Management of NTRK-altered pDLGG -- 8 Conclusion -- References -- Chapter Two: Tumor-specific biology, diagnosis, and therapy -- 1 Introduction -- 2 Tumor-specific biology -- 2.1 Molecular features -- 2.1.1 H3K27M-mutant DMG -- 2.1.2 Other genetic subtypes of pHGG -- 2.2 Epigenetic and transcriptomic features -- 2.2.1 Diffuse midline glioma, H3K27-altered -- 2.2.2 Diffuse hemispheric glioma, H3 G34-mutant -- 2.2.3 Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype -- 2.2.4 Infant-type hemispheric glioma -- 2.3 Tumor microenvironment and immunobiology -- 2.3.1 Immune infiltrates in pHGG and DMG -- 2.3.2 Blood-brain barrier and immune evasion.
2.3.3 Myeloid-dominant tumor milieu and tumor-associated macrophages/microglia -- 2.4 Developmental context -- 3 Diagnosis and classification -- 3.1 Classification systems -- 3.2 Diagnostic imaging -- 3.3 Histopathology and immunohistochemistry -- 3.4 Molecular diagnostics -- 4 Therapeutic strategies -- 4.1 Standard-of-care treatment -- 4.2 Targeted therapies -- 4.2.1 Histone-targeted therapies -- 4.2.2 Pathway-specific targeted therapies -- 4.2.3 Challenges in targeted therapy development -- 4.2.4 Immunotherapy -- 4.2.5 Vaccine approaches -- 4.2.6 Immune checkpoint inhibitors -- 4.2.7 CAR T-cell therapy -- 4.2.8 Oncolytic virus therapy -- 4.2.9 Adoptive cell therapy -- 4.3 Novel drug delivery approaches -- 4.3.1 Convection-enhanced delivery (CED) -- 4.3.2 Nanoparticle and liposome delivery systems -- 4.3.3 Focused ultrasound (FUS) -- 4.3.4 Intranasal and intrathecal delivery -- 4.3.5 Targeted antibody-drug conjugates (ADCs) -- 4.4 Clinical trials and translational research -- 4.4.1 Landscape of current and recent clinical trials -- 4.4.2 Basket and umbrella trial designs -- 4.4.3 Challenges in trial enrollment and biomarker selection -- 4.4.4 Ongoing translational gaps and future trial design -- 5 Challenges and future directions -- 6 Conclusions -- References -- Chapter Three: Three decades of radiotherapy advancements for pediatric ependymoma -- 1 Radiotherapy -- 2 The radiation oncologist's perspective -- 3 North American experience -- 4 European experience -- 5 Radiotherapy planning -- 6 Reirradiation -- 7 Summary -- References -- Chapter Four: An overview of the diagnosis and management of Choroid Plexus tumors -- 1 Introduction -- 2 Choroid plexus papilloma -- 3 Atypical choroid plexus papilloma -- 4 Choroid plexus carcinoma -- 4.1 Epidemiology -- 4.2 Imaging characteristics -- 4.3 Pathology.
4.4 Li Fraumeni syndrome-associated CPC and molecular testing -- 4.5 Surgery -- 4.6 Embolization -- 4.7 Conventional chemotherapies -- 4.8 Myeloablative chemotherapy with stem cell transplant -- 4.9 Radiation therapy -- 4.10 Preclinical data and novel therapeutic strategies -- 4.11 Future trials of therapy for CPC -- 5 Conclusion -- Acknowledgements -- References -- Chapter Five: Medulloblastoma chapter -- past perspectives and future directions -- 1 Introduction -- 2 Clinical presentation -- 2.1 Genetic predisposition -- 3 Diagnostics -- 3.1 Advances in imaging -- 3.1.1 Advances in artificial intelligence (AI) -- 3.2 Advances in molecular testing -- 3.2.1 Histopathology -- 3.2.2 Tumor-specific biology -- 3.2.3 Wingless/INT1 (WNT) subtype -- 3.2.4 Sonic Hedgehog (SHH) subtype -- 3.2.5 Group 3/4 (Non-WNT/Non-SHH) -- 3.2.6 Tumor microenvironment -- 4 Risk stratification -- 4.1 Liquid biopsy -- 5 Therapeutic advances -- 5.1 Surgical therapy -- 5.1.1 Goals of surgery -- 5.2 Surgical techniques -- 5.2.1 Preoperative preparation -- 5.2.2 Key surgical approaches to the posterior Fossa -- 5.2.3 Advances in surgical technology -- 5.3 Radiation therapy -- 5.4 Chemotherapy -- 5.5 Special group: Medulloblastoma in infants and young children (iMB) -- 5.6 Targeted therapies -- 6 Relapsed medulloblastoma (rMB) -- 7 Survivorship -- 7.1 Acute complications -- 7.2 Late-effects -- 7.2.1 Neurocognitive effects -- 7.2.2 Second malignant neoplasms (SMNs) -- 7.2.3 Endocrinopathies -- 7.2.4 Ototoxicity -- 7.2.5 Cerebrovascular disease -- 8 Future directions and challenges -- 9 Conclusion -- References -- Chapter Six: Current advances in the management of atypical teratoid rhabdoid tumors (ATRT) -- 1 Introduction -- 2 Epidemiology and clinical presentation -- 2.1 Rhabdoid tumor predisposition syndrome -- 3 Molecular pathogenesis -- 3.1 SMARCB1/SMARCA4 inactivation.
3.2 Molecular subgroups of ATRT -- 3.2.1 ATRT-TYR -- 3.2.2 ATRT-SHH -- 3.2.3 ATRT-MYC -- 3.3 Impact of molecular subgroups on prognosis -- 3.4 Current conventional therapeutic approaches -- 3.4.1 EU-RHAB -- 3.4.2 DFCI-ATRT -- 3.5 Head Start trials -- 3.6 St. Jude trials -- 3.7 ACNS0333 -- 3.8 Prognostic impact of specific treatment modalities for ATRT -- 3.8.1 Impact of extent of resection -- 3.8.2 Impact of radiation therapy (RT) -- 3.8.3 Impact of high dose chemotherapy with autologous stem cell rescue (HDCASCR) -- 3.8.4 Impact of intrathecal/intraventricular chemotherapy -- 3.9 Summary recommendations based on prospective upfront ATRT clinical trials -- 4 Innovative mechanism-driven therapeutics for ATRT -- 4.1 Chromatin remodeling and histone modification -- 4.1.1 HDAC inhibition -- 4.1.2 EZH2 inhibition -- 4.1.3 Bromodomain and extra-terminal protein (BET) inhibition -- 4.1.4 Other epigenetic therapies -- 4.2 Cell cycle regulation -- 4.2.1 Aurora A kinase inhibition -- 4.2.2 Cyclin D1 inhibition -- 4.2.3 MDM2 inhibition -- 4.2.4 XPO1 blockade -- 4.3 Other associated signaling pathways -- 4.3.1 PI3K pathway inhibition -- 4.3.2 MAPK pathway inhibition -- 4.3.3 LIN28/Let-7 pathway inhibition -- 4.3.4 Other signal transduction pathway inhibitors -- 4.4 Integrated stress response (ISR) -- 4.4.1 Proteasome inhibition -- 4.4.2 Anti-apoptotic inhibition -- 4.4.3 Metabolic targeting -- 5 Promise of immunotherapeutic strategies for ATRT -- 5.1 Immune checkpoint inhibition -- 5.2 Combined epigenetic dysregulation and immune checkpoint inhibition -- 5.3 Vaccine-based therapy -- 5.4 Oncolytic viral therapy -- 5.5 Radioimmunotherapy -- 5.6 Chimeric antigen receptor (CAR) therapy -- 6 Summary -- References -- Chapter Seven: Craniopharyngioma -- 1 Introduction -- 2 ACP tumor biology -- 2.1 Tissue origins -- 2.2 Histological characteristics.
3 Diagnosis of adamantinomatous craniopharyngioma -- 3.1 Signs and symptoms at presentation -- 3.2 Diagnostic imaging -- 4 Therapy -- 4.1 Surgery -- 4.1.1 Goals of surgery -- 4.1.2 Cyst-directed surgery -- 4.1.3 Operative approaches -- 4.2 Radiation therapy -- 4.3 Complications related to tumor and therapy -- 4.3.1 Endocrinopathies -- 4.3.2 Visual deficits -- 4.3.3 Future directions and developmental therapeutics in clinical trials -- 5 Conclusion -- References -- Chapter Eight: Germinoma: Presentation, Management, and Recent Advances -- 1 Introduction -- 2 Epidemiology and genetic predisposition -- 3 Clinical presentation -- 4 Pathogenesis -- 5 Histopathology -- 6 Diagnostic and staging evaluations -- 6.1 Neuroimaging -- 6.2 Tumor markers -- 6.3 Histopathologic and cytologic examination -- 7 Treatment strategies -- 7.1 Emergency management -- 7.2 Surgery -- 7.3 Adjuvant therapy -- 7.4 The European experience -- 7.5 The North American and Australian experience -- 7.6 The East Asian experience -- 7.7 Basal ganglia and metastatic germinoma -- 7.8 Relapsed germinoma -- 8 Recent advances and future directions -- 9 Conclusion -- Acknowledgement -- References -- Chapter Nine: Non-germinomatous germ cell tumors of the CNS: Classification, diagnosis, and treatment -- 1 Introduction -- 2 Clinical presentations -- 2.1 Diagnosis -- 2.2 Tumor marker -- 2.3 Imaging -- 2.4 Cerebrospinal fluid cytology -- 2.5 Histopathological features -- 3 Treatment strategy -- 3.1 Initial management and biopsy strategy -- 3.2 Chemotherapy and radiation therapy -- 3.3 Surgery -- 3.4 Management of growing teratoma syndrome during NGGCT treatment -- 3.5 Follow-up -- 3.6 Relapse -- 4 Tumor-specific biology -- 4.1 Genomic abnormalities -- 4.2 Cellular origins and differentiation -- 4.3 Epigenetic features and tumorigenesis -- 4.4 Comparison with testicular germ cell tumors.
4.5 Genetic factors underlying ethnic differences in CNS GCT incidence.