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The practice of neuro-oncology entails the management of many different types of tumors of the central and peripheral nervous system by multidisciplinary teams of healthcare providers. These tumors represent a diverse spectrum of underlying molecular subtypes, prognostic categories, age distributions, and treatment recommendations. The World Health Organization (WHO) classification of central nervous system tumors is the foundation for the categorization and, by extension, clinical management and treatment of patients with all types of nervous system tumors. The WHO classification had traditionally been based on light microscopic description of the cellular elements of tumors in the brain, spinal cord, nerves, and meninges. The 2016 WHO classification of central nervous system tumors for the first time incorporated molecular markers into the categorization of some types of nervous system tumors, particularly gliomas. The 2021 WHO classification took another big step forward to dissect many entities based on molecular profiling, resolving the hitherto unclear separation of isocitrate dehydrogenase (IDH)-mutant versus IDH-wildtype tumors, and by attributing a major diagnostic role to genomic methylation profiling across tumor groups. This revised classification serves as the updated basis for patient prognostication, clinical trial eligibility, and management recommendations for these newly recognized pathological–molecular subsets of central nervous system tumors. Many current management guidelines are derived, however, from clinical trials and studies utilizing earlier versions of the WHO classification system. This book is intended for clinicians as a complement to the WHO classification system with a focus on clinical management of nervous system tumors in adults and children. Each chapter in the book is numbered to mirror the corresponding entity in the 2021 WHO classification system. Each chapter is co-authored by a multidisciplinary, international group of leading authorities in adult and pediatric neuro-oncology. The book is organized according to the 2021 WHO classification of central nervous system tumors and each chapter follows a similar framework. The introductory chapter reviews the 2021 revision of the WHO classification of central nervous system tumors and how these changes may influence clinical trials, clinical practice, and subsequent editions of this book.
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