Genetic Syndromes Associated with CNS Tumors

Central nervous system (CNS) tumors represent a rare group of malignancies associated with significant morbidity and mortality across all age groups. The highest incidence of CNS malignancies occurs in the pediatric population, particularly among children aged 0–14 years. CNS tumors constitute approximately 20–25% of all childhood malignancies. The principal etiological factors implicated in the development of CNS tumors include inherited genetic syndromes and exposure to ionizing radiation. This chapter focuses on a detailed review of the genetic syndromes associated with CNS tumors, with emphasis on their diagnostic criteria.