Ependymomas

Ependymomas are glial tumors that arise from ependymal cells lining the ventricular system and the central canal of the spinal cord. They account for a minority of primary brain tumors in children and are among the more common intramedullary spinal tumors in adults. In children, they most often occur in the fourth ventricle; in adults, spinal ependymomas are more frequent.

Histologically they are classified by the World Health Organization as classic ependymoma (grade II), anaplastic ependymoma (grade III); myxopapillary ependymoma (grade I) occurs most often in the cauda equina/conus; subependymoma is a separate, usually indolent entity. Typical microscopic features include perivascular pseudorosettes and, less commonly, true ependymal rosettes.

Clinical presentation depends on location. Intracranial tumors may cause hydrocephalus with headaches, nausea, and vomiting; spinal tumors cause back pain, progressive weakness, sensory changes, and bladder or bowel dysfunction.

MRI with gadolinium is the imaging modality of choice, showing a well-demarcated mass that typically enhances variably. Intraventricular and intramedullary spinal ependymomas may have cysts or calcifications; CT can help detect calcifications.

Management centers on maximal safe surgical resection. Gross total resection is associated with improved control, particularly for classic and spinal ependymomas. Adjuvant radiotherapy is commonly used for residual tumor or higher-grade lesions; chemotherapy has limited proven role. Prognosis varies with age, tumor site, histology, and extent of resection, with spinal ependymomas generally having a favorable outcome compared with many intracranial counterparts and requiring long-term follow-up due to recurrence risk.