Abstract
Ependymal tumors—ependymoma, subependymoma, and myxopapillary ependymoma—represent a broad group of gliomas that manifests morphologic and ultrastructural evidence of ependymal differentiation. They account for 5% to 9% of all primary brain tumors in adults, 6% to 12% in children, and up to 30% in infants. Ependymal tumors are considered to originate from radial glial-like stem cells lining the wall of the ventricles or the wall of the spinal canal, which is why they can occur along the entire neuroaxis. Because of advanced molecular testing techniques such as DNA methylation profiling, categorization of ependymomas has completely changed in the 2021 WHO classification from being based on morphology to being based on location and molecular alteration into molecular groups across the supratentorial, posterior fossa, and spinal compartments. Two molecularly defined types of supratentorial ependymoma (with ZFTA or YAP1 fusion), two molecularly defined types of posterior fossa ependymoma (PFA and PFB), and a spinal ependymoma defined by the presence of MYCN amplification are now listed in this new classification scheme. This chapter reviews the clinicopathological features and differential diagnosis of all these ependymoma types as well as of their histological variants.
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Ependymoma
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Myxopapillary Ependymoma
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Subependymoma
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Lacruz, C.R. (2023). Ependymal Tumors. In: Lacruz, C.R. (eds) Central Nervous System Tumors . Springer, Cham. https://doi.org/10.1007/978-3-031-51078-6_7
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DOI: https://doi.org/10.1007/978-3-031-51078-6_7
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