Ependymoma
Ependymoma is a central nervous system tumor arising from the ependymal cells lining the brain's ventricles and the central canal of the spinal cord. The 2021 World Health Organization (WHO) classification divides ependymomas into subgroups based on anatomical location and molecular features. Complete surgical resection is the most important prognostic factor in treatment.
Última atualização: 2026-06-06
Definition
Ependymoma is a primary central nervous system tumor that develops from the ependymal cells lining the brain's ventricles and the central canal of the spinal cord. It accounts for roughly 3% of all central nervous system tumors and can arise in both the brain and the spinal cord.
Epidemiology
Ependymoma has a bimodal age distribution. In children it usually occurs at younger ages and is often located in the posterior fossa; in adults a spinal (spinal cord) location is more common. Spinal ependymomas make up a significant proportion of primary spinal cord tumors in adults. Neurofibromatosis type 2 (NF2) particularly increases the risk of spinal ependymoma.
Symptoms
Symptoms depend on tumor location. Posterior fossa tumors may cause hydrocephalus with headache from raised intracranial pressure, morning vomiting, balance and coordination problems (ataxia) and double vision; infants may show head enlargement and irritability. Spinal ependymomas cause pain, weakness and sensory loss according to the level of the tumor.
Diagnosis
Contrast-enhanced brain MRI is the principal diagnostic method. Evaluation of the entire neuraxis is important; therefore brain and whole-spine MRI are performed, together with cerebrospinal fluid (CSF) cytology in cases at risk of leptomeningeal (meningeal) spread. The CSF sample is usually taken several weeks after surgery to avoid false positives. Definitive diagnosis is made by pathological examination; the 2021 WHO classification incorporates methylation profiling and specific molecular markers.
Classification and Grading (2021 WHO CNS5)
The 2021 WHO classification divides ependymomas into subgroups based on anatomical location (supratentorial, posterior fossa, spinal) and molecular features. For example, supratentorial ependymomas are categorized by ZFTA or YAP1 fusion status, and posterior fossa ependymomas into PFA and PFB groups by methylation profile. These molecular subtypes directly affect prognosis and treatment decisions; some subgroups (such as PFB and most spinal ependymomas) behave more favorably, while others (PFA) are more aggressive.
Treatment Options
Surgery is the most critical step in ependymoma; complete (gross total) resection is the strongest prognostic factor and markedly influences survival. Hydrocephalus may require a shunt or endoscopic third ventriculostomy. After surgery, focal radiotherapy may be given according to tumor grade, molecular subtype and extent of resection; craniospinal radiotherapy is required in the presence of leptomeningeal spread. The role of chemotherapy is limited and is mainly used to delay radiotherapy in very young children or in recurrent disease. In children, proton therapy may be preferred because it spares normal tissue.
Prognosis
Prognosis varies markedly with the extent of resection, tumor grade, age and molecular subtype. Outcomes are better in cases with complete resection and in favorable subtypes (such as most spinal ependymomas). Long-term MRI follow-up is important in all patients, because recurrence may appear years later. Outcomes vary individually and no outcome can be guaranteed.
Referências
- Greenberg MS. Greenberg's Handbook of Neurosurgery. 10th ed. Thieme; 2023:724.
- Osborn AG, Hedlund GL, Salzman KL. Osborn's Brain: Imaging, Pathology, and Anatomy. 2nd ed. Elsevier; 2018:560.
- Louis DN, et al. The 2021 WHO Classification of Tumors of the Central Nervous System. Neuro Oncol. 2021.
- Rudà R, et al. EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro Oncol. 2018.
Este artigo é informativo e não substitui um exame médico. As decisões de diagnóstico e tratamento são individuais.