Provided by Genetics of Ependymoma Lead contributors: Miriam Kimpo, MD Cardinal Santos Memorial Hospital Manila, Philippines A. Epidemiology Ependymomas are glial tumors arising from the ependymal lining of the ventricles and spinal canal. They account for 9% of all neuroepithelial tumors and have a mean prevalence of 1.7–3% among primary central nervous system (CNS) tumors. 1,2 In children and young adults, ependymomas are the third most common primary brain tumors, accounting for 6–12% of all such tumors. with 50% of cases involving children younger than five years of age. . 3,4,5,6 Of these tumors, 90% are located intracranially with two thirds found infratentorially. 1,6 The annual incidence is 5.8 7 and is higher in boys with the sex ratio of 1.33 to 2.1. 8 The age-specific incidence rate average is 8.9 per million in the first 4 years of life and declines thereafter. The 5-year event-free survival rate ranges from 50% to 62%, 7,3 and the estimated 10-year survival rate is 53%. 9 In the World Health Organization (WHO) classification system, ependymomas are categorized as grade I myxopapillary ependymomas and subependymomas, grade II ependymomas, and grade III anaplastic ependymomas. The latter 2 types are predominant in children. 4 Local tumor recurrence with progression is the most common cause of death in patients with intracranial lesions.
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