title: Ependymoma: a heterogeneous tumor of uncertain origin and limited therapeutic options.
abstract: Ependymomas are tumors that typically occur with an age-based site preference, with adults harboring supratentorial and spinal tumors and pediatric tumors being mainly in the posterior fossa. Despite their similar histologic appearance, the prognosis varies significantly by age and tumor location, with a better prognosis in increasing age. The mainstay of treatment remains surgical excision with or without radiation therapy as the tumor biology is poorly understood and chemotherapy is generally considered to be ineffective. More recently, molecular biology data have increased our understanding of the genetic and epigenetic changes that drive these tumors, but still it will take a lot of effort to find effective chemotherapeutic regimens. Currently, we are trying to define a subset of tumors, for which radiation therapy can be avoided.
authors.affiliation: Division of Neurosurgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada; Department of Neurosurgery, Medical University of Vienna, Vienna, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Dorfer
authors.affiliation: Department of Neurosurgery, University Clinic of Ludwig-Maximilians-University Munich-Großhadern, Munich, Germany.
authors.forename: Joerg
authors.initials: J
authors.lastname: Tonn
authors.affiliation: Division of Neurosurgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada. Electronic address: james.rutka@sickkids.ca.
authors.forename: James T
authors.initials: JT
authors.lastname: Rutka
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy; D004806:Ependymoma / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy*; D006801:Humans; D059906:Neuroimaging; D057089:Pathology, Molecular
keywords: ependymoma; intraventricular tumors; myxopapillary ependymoma; pediatric posterior fossa tumors; spinal tumors; subependymoma