ependimomas

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 central nervous system tumors, with a higher incidence in children than in adults. Ependymomas can develop anywhere along the ventricular system or within the spinal canal.

Most intracranial ependymomas occur in the fourth ventricle, where they can cause obstructive hydrocephalus. Spinal ependymomas commonly arise in the filum terminale or within the spinal cord itself. Histologically, ependymomas are classified by the World Health Organization into grades 1–3, with variants including myxopapillary ependymoma (often considered grade 2) and subependymoma (grade 1); anaplastic ependymoma is grade 3 and more aggressive.

Clinical presentation varies by location. Intracranial tumors may present with headaches, nausea, vomiting, ataxia, or signs of increased intracranial pressure. Spinal ependymomas typically cause back pain, weakness, sensory changes, and gait disturbances due to compression or invasion of spinal pathways.

Diagnosis relies primarily on magnetic resonance imaging with contrast to define the tumor and its extent. Computed tomography can be used in some cases. Definitive diagnosis requires histopathologic examination after biopsy or surgical resection. CSF cytology may show tumor cells but is not a definitive diagnostic test.

Treatment centers on surgery to achieve maximal safe resection. Gross total resection is associated with better outcomes. Adjuvant radiotherapy is commonly recommended for residual disease or higher-grade tumors. Chemotherapy has a limited role, mainly in very young children or in recurrent disease. Prognosis depends on tumor grade, location, and extent of resection; lower-grade tumors and complete resections tend to have better long-term control, while higher-grade tumors have a more guarded prognosis.