Pediatric Cancer, Volume 3: Diagnosis, Therapy, and Prognosis (Pediatric Cancer, 3)

Brain tumors are the most common solid tumor of childhood as well as the leading cause of cancer-related mortality in children. This third volume of the Springer series discussing pediatric cancer focuses on diagnosing, treating, and assessing the future course of malignant brain neoplasms in children. In addition to a general introduction to the principals involved, the material includes vital research in molecular genetics, a major contribution to the molecular characterization of solid tumors, which will define new biomarkers of the disease and identify molecular pathways. Now it has become possible to achieve the goal of targeting new, more effective therapies to minimize the tragedy faced by children. This research features the application of molecular genetics in combating atypical teratoid/rhabdoid tumor (AT/RT), a highly aggressive embryonal CNS tumor that is among the most common malignant neoplasms in children, with a peak occurrence in infants younger than three years old. Contributors also examine the reasons for the common misdiagnosis of AT/RT tumors as other types of CNS tumors. They help resolve this issue by indicating the details of an abnormality in the genetics of AT/RT, which is unique to AT/RT type. The INII gene on chromosome 22q11 is involved in the AT/RT. The presence of this gene and the SMARCB 1 is discussed. In addition to discussing these important topics, the volume includes presentations of present and future therapies. The volume also explains AT/RT’s dissemination to the cerebral fluid, the molecular mechanisms underlying the progression of medulloblastoma, and the importance of gamma knife radiosurgery during multimodality management of medulloblastoma/PNET tumors. Other topics discussed include using magnetic resonance imaging for diagnosing retinoblastoma, and mapping the effects of radiotherapy in low-grade glioma in children. Information on alterations in cell-cycle regulators that are influenced by tumor suppressor genes and oncogenes is detailed. Contributors provide recommendations concerning non-narcotic analgesic routines for children recovering from cranial and spinal surgery. The practical knowledge of frontier-expanding research presented leads this authoritative volume to be a compelling addition to the literature. Front Matter....Pages i-xxxi Front Matter....Pages 1-1 Early Childhood Atypical Teratoid/Rhabdoid Tumors....Pages 3-12 Atypical Teratoid/Rhabdoid Tumors....Pages 13-24 Paediatric Teratoid/Rhabdoid Tumours: Germline Deletions of Chromosome 22q11.2....Pages 25-34 Pediatric Atypical Teratoid/Rhabdoid Tumor: Role of INI1 Tumor Suppressor Gene....Pages 35-44 Atypical Teratoid/Rhabdoid Tumors: Diagnosis Using Imaging....Pages 45-53 Pediatric Atypical Teratoid/Rhabdoid Tumor in the Spine: Diagnosis and Treatment....Pages 55-62 Early Childhood Clival-C2 Atypical Teratoid/Rhabdoid Tumor: Gross Total Resection Followed by Aggressive Chemotherapy and Radiation....Pages 63-69 Pediatric Atypical Teratoid/Rhabdoid Tumors: Dissemination to the Cerebrospinal Fluid....Pages 71-74 Front Matter....Pages 75-75 Teratoid Pediatric Medullomyoblastoma....Pages 77-80 Pediatric Medulloblastoma: Mechanisms of Initiation and Progression....Pages 81-91 Medulloblastoma Initiation and Growth: Role of Hepatocyte Growth Factor....Pages 93-101 Medulloblastoma – Molecular Genetics....Pages 103-116 Pediatric Medulloblastoma: The Role of Heterozygous Germ-Line Mutations in the NBN Gene....Pages 117-127 Pediatric Medulloblastoma: Pituitary Adenylyl Cyclase Activating Peptide/Protein Kinase A Antagonism of Hedgehog Signaling....Pages 129-135 Medulloblastoma: Role of MYCN Gene Amplification Using Fluorescence In Situ Hybridization and Real Time Quantitative PCR Methods....Pages 137-144 Pediatric Medulloblastoma: Ultrastructure....Pages 145-151 Pediatric Medullomyoblastoma: Immunohistochemical Analyses....Pages 153-158 Boost Gamma Knife Radiosurgery During Multimodality Management of Medulloblastoma/PNET Tumors....Pages 159-171 Early Childhood Medulloblastoma: Prognostic Factors....Pages 173-181 Front Matter....Pages 183-183 Trilateral Retinoblastoma: Diagnosis Using Magnetic Resonance Imaging....Pages 185-193 Front Matter....Pages 183-183 Pediatric Intraocular Retinoblastoma: Treatment....Pages 195-201 Front Matter....Pages 203-203 Pediatric High-Grade Glioma: Role of Microsatellite Instability....Pages 205-210 Low-Grade Glioma in Children: Effects of Radiotherapy....Pages 211-217 Front Matter....Pages 219-219 Pediatric Multiple Primary Cranio-Spinal Tumors Associated with Neurofibromatosis Type 2: Combined Therapeutical Strategies....Pages 221-229 Pediatric Intradural Lipoma of the Cervicothoracic Spinal Cord: Laminectomy and Duraplasty....Pages 231-241 Front Matter....Pages 243-243 Pediatric Embryonal Tumors: Prognostic Role of Cyclin A and B1 Proteins....Pages 245-249 Intracranial Pediatric Ependymoma: Role of Cytogenetic Markers Using Comparative Genomic Hybridization and Fluorescent In Situ Hybridization....Pages 251-262 Pediatric Hepatoblastoma. Complete Surgery and Ultrasound Monitoring....Pages 263-274 Treatment of Pineal Region Tumors in Childhood....Pages 275-280 Benign Testicular Tumors in Children: Testicular Preserving Surgery....Pages 281-287 Erratum....Pages E1-E1 Back Matter....Pages 289-301 Brain tumors are the most common solid tumor of childhood as well as the leading cause of cancer-related mortality in children. This third volume of the Springer series discussing pediatric cancer focuses on diagnosing, treating, and assessing the future course of malignant brain neoplasms in children. In addition to a general introduction to the principals involved, the material includes vital research in molecular genetics, a major contribution to the molecular characterization of solid tumors, which will define new biomarkers of the disease and identify molecular pathways. Now it has become possible to achieve the goal of targeting new, more effective therapies to minimize the tragedy faced by children. This research features the application of molecular genetics in combating atypical teratoid/rhabdoid tumor (AT/RT), a highly aggressive embryonal CNS tumor that is among the most common malignant neoplasms in children, with a peak occurrence in infants younger than three years old. Contributors also examine the reasons for the common misdiagnosis of AT/RT tumors as other types of CNS tumors. They help resolve this issue by indicating the details of an abnormality in the genetics of AT/RT, which is unique to AT/RT type. The __INII__ gene on chromosome 22q11 is involved in the AT/RT. The presence of this gene and the SMARCB1 is discussed. In addition to discussing these important topics, the volume includes presentations of present and future therapies. The volume also explains AT/RT’s dissemination to the cerebral fluid, the molecular mechanisms underlying the progression of medulloblastoma, and the importance of gamma knife radiosurgery during multimodality management of medulloblastoma/PNET tumors. Other topics discussed include using magnetic resonance imaging for diagnosing retinoblastoma, and mapping the effects of radiotherapy in low-grade glioma in children. Information on alterations in cell-cycle regulators that are influenced by tumor suppressor genes and oncogenes is detailed. Contributors provide recommendations concerning non-narcotic analgesic routines for children recovering from cranial and spinal surgery. The practical knowledge of frontier-expanding research presented leads this authoritative volume to be a compelling addition to the literature.