Clinical manifestations of childhood ependymoma: a multitude of syndromes.

Over 90% of childhood ependymomas arise within the cranium, two-thirds below and one-third above the tentorium, and they comprise 8-10% of all childhood CNS neoplasms. This is in contradistinction to the presentation in adults where over 75% of the ependymomas arise within the spinal canal. The incidence of 2.2 cases per million appears to be increasing over the past 30 years. The biology of the disease resembles that of a low-grade glioma where local control measures are most important and less than 5% of children present with metastatic disease. Thus, total resection is the optimum therapy. The value of adjuvant therapy for children with no postoperative residual disease is unclear. Adjuvant radiotherapy is reserved for children with postoperative residual disease and appears to prolong survival. A brief review of our current understanding of the incidence, sites of origin, clinical presentations, prognostic factors and controversial treatment issues will be presented.