Ependymal tumors

Opinion statement

Ependymal tumors are rare malignancies that arise from the cells that line the ventricles and central canal of the spinal cord. Although they are more common in children, adults may also be effected by ependymal tumors. Prognosis is dependent on tumor location, histology, especially for myxopapillary tumors that tend to occur in the lumbar spine, extent of surgical resection, and stage of disease. Standard therapy consists of complete resection when feasible. The exact role of adjuvant radiotherapy in patients with radiographically confirmed complete resection is poorly defined. Patients with known residual disease may benefit from local radiation therapy, but the extent of radiation field and total dose are controversial. Even in patients treated with involved field radiotherapy, most relapses occur within the original tumor bed, thus local control remains the biggest obstacle to effective therapy. Chemotherapy has little impact against this tumor and has no role in the adjuvant setting, outside of a well designed clinical trial, with the possible exception of children younger than 5 years in an effort to delay radiation. A minority of patients may respond to one of several chemotherapy regimens at the time of recurrence, but the impact of this therapy is limited. Newer treatment strategies are needed.