Ependymomas are gliomas that appear to originate from the lineage of cells that give rise to the differentiated ependymal cell layer lining the ventricular system and central canal of the spinal cord. Intracranial ependymomas comprise approximately 9% of all brain tumors in patients under 20 years of age, and are the third most common primary brain tumor in children (following astrocytomas and primitive neuroectoder-mal tumors). Data from the SEER group (Surveillance, Spidemiology, and End Results) suggest that the annual incidence of ependymoma is 2.6 per million for the 0–14 age group, and 2.2 per million for the 0–20 age group (Linet et al. 1999; Ries et al. 1999). The Connecticut Tumor Registry reports that between 1935 and 1973, 5 spinal cord ependymomas and 44 intracranial ependymomas were identified in the Connecticut population under 20 years of age, suggesting that spinal cord ependymoma represents approximately 10% of all ependymal tumors in children and young adults (Dohrmann et al. 1976). Another large institutional series confirmed that spinal cord ependymomas are rare in children under 10 years, comprising less than 1% of all spinal tumors. After the age of 10, the incidence of spinal cord ependymoma increases and it represents the majority of intramedullary tumors in patients older than 20 years (Constantini et al. 1997).
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Smyth, M.D., Rubin, J. (2010). Ependymoma. In: Gupta, N., Banerjee, A., Haas-Kogan, D. (eds) Pediatric CNS Tumors. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-87979-4_4
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