TY - BOOK AU - Karajannis,Matthias A. AU - Allen,Jeffrey TI - Neurofibromatosis type 1: symptoms, treatment and prognosis T2 - Genetics-Research and issues SN - 9781628081657 PY - 2013///] CY - New York PB - Nova Biomedical KW - Neurofibromatosis KW - Neurofibromatosis in children N1 - Includes bibliographical references and index; NEUROFIBROMATOSIS TYPE 1 ; Library of Congress Cataloging-in-Publication Data; Contents ; Acknowledgment ; Introduction ; Chapter I Molecular Pathogenesis of Neurofibromatosis Type 1 ; Abstract ; Introduction ; Epidemiology of NF1 ; Clinical Features of NF1 ; Skin Manifestations ; Skeletal and Osseous Abnormalities ; Vascular Lesions ; Psychiatric Disorders and Cognitive Dysfunction; Rare Manifestations ; RASopathies ; Dysplasia and Developmental Defects ; Molecular Genetics of NF1 ; Genetic Diagnosis ; Tumors Associated with NF1 ; Disease Modifier Genes in NF1; Molecular Pathogenesis of NF1Using Systems Approaches to Study NF1 ; Current Treatment and Disease Management ; Conclusion ; References ; Chapter II Imaging of Plexiform Neurofibromas in Neurofibromatosis Type 1 ; Abstract ; Introduction ; Imaging Appearance of Plexiform Neurofibromas ; Plexiform Neurofibroma Types ; Volumetric Analysis ; Cranial Nerves ; Spinal Nerves ; Neck, Mediastinum, Chest ; Abdomen, Pelvis ; Malignant Peripheral Nerve Sheath Tumors (MPNSTs) ; Conclusion ; References ; Chapter III Surgical Treatment of GiantNeurofibromas ; Abstract ; Introduction; Surgical Techniques Discussion ; Conclusion ; References ; Chapter IV Neurofibromatosis Type 1-Associated Nervous System Tumors and Current Treatment Strategies ; Abstract ; Introduction ; Central Nervous System Tumors in Children with NF1 ; Low Grade Glioma: Clinical Presentation ; Low Grade Glioma: Imaging Characteristics ; Low Grade Glioma: Prognosis ; Low Grade Glioma: Treatment Modalities ; 1. Role of Surgery ; 2. Radiation Therapy ; 3. Chemotherapy; 4. Targeted Therapy ; RAS-MAPK Pathway; Peripheral Nervous System Tumors in Children with NF1; Plexiform Neurofibromas: Clinical Presentation Plexiform Neurofibromas: Imaging Characteristics ; Plexiform Neurofibromas: Prognosis ; Plexiform Neurofibromas: Treatment Modalities ; 1. Role of Surgery ; 2. Radiation Therapy ; 3. Chemotherapy ; 4. Targeted Therapy ; a) mTOR Pathway ; Interferon Treatment ; b) Anti-angiogenic Treatment ; c) Tyrosine Kinase Inhibitor Treatment ; Malignant Peripheral Nerve Sheath Tumor (MPNST): Clinical Presentation ; MPNST: Imaging Characteristics ; MPNST: Prognosis ; MPNST: Treatment Modalities ; 1. Role of Surgery ; 2. Radiation Therapy ; 3. Chemotherapy; 4. Targeted Therapy a) Tyrosine Kinase Inhibitor Treatment; b) Angiogenic Pathways ; c) Cytoskeletal Remodeling ; Conclusion ; References ; Chapter V Outcome Measures for Optic Pathway Gliomas ; Abstract ; Introduction ; Epidemiology of OPGs ; Presentation of OPGs ; Clinical Symptoms ; Diagnostic Imaging ; Pathology ; Management of OPGs ; Clinical Course ; Surveillance ; Treatment ; Outcome Measures ; Anatomical Outcomes (MRI) ; Visual Outcomes ; Alternative Outcome Measures ; Diffusion and Perfusion Imaging; Diffusion Tensor Imaging ; Positron Emission Tomography UR - https://search.ebscohost.com/login.aspx?custid=ns123844&authtype=ip,shib&direct=true&scope=site&db=nlebk&db=nlabk&AN=620125 ER -