What are the clinical features of ependymoma (a type of central nervous system tumor)?

From the Guidelines

Ependymomas are characterized by clinical features that vary based on their location within the central nervous system, including symptoms such as headaches, nausea, vomiting, and dizziness due to increased intracranial pressure, as reported in the 2018 European Association of Neuro-Oncology guidelines 1. The clinical presentation of ependymomas can be diverse, depending on the tumor location, with common symptoms including:

• Headaches
• Nausea
• Vomiting
• Dizziness
• Seizures
• Visual disturbances
• Balance problems
• Neck pain Spinal ependymomas typically cause:
• Back pain
• Sensory changes
• Weakness
• Bowel or bladder dysfunction In children, symptoms may include:
• Irritability
• Lethargy
• Developmental delays
• Failure to thrive The onset of symptoms is usually gradual, worsening over weeks to months, and physical examination may reveal:
• Papilledema
• Cranial nerve palsies
• Motor weakness
• Sensory deficits
• Abnormal reflexes Diagnosis requires neuroimaging, preferably MRI with contrast, showing a well-defined, heterogeneously enhancing mass, often with cystic components, followed by histopathological confirmation after surgical resection, as recommended in the 2018 guidelines 1. Infratentorial tumors (posterior fossa) often cause hydrocephalus, cranial nerve deficits, and cerebellar signs like ataxia and nystagmus, while supratentorial tumors may lead to focal neurological deficits, personality changes, and cognitive impairment, highlighting the importance of prompt diagnosis and treatment, as emphasized in the 2018 guidelines 1.

From the Research

Clinical Features of Ependymoma

The clinical features of ependymoma, a type of central nervous system tumor, vary depending on the location and age of the patient.

• Ependymomas can occur in all age groups, but the location of the tumor differs by age:
  • In adults, ependymomas are more common in the spinal cord 2
  • In children, ependymomas are more common in the brain, particularly in the posterior fossa 3
• The prognosis of ependymoma also varies by age and tumor location, with a better prognosis in older patients 3
• Clinical features can include a range of symptoms depending on the tumor location, such as neurological deficits or spinal cord compression symptoms 2

Treatment and Outcome

The main treatment for ependymoma is maximal surgical resection, often followed by radiation therapy.

• Gross total resection is considered the first choice in treatment of spinal ependymomas, and the most important predictor of clinical outcome is preoperative neurologic functioning 2
• The role of postoperative radiation therapy is still unclear, particularly for low-grade ependymomas 2, 4
• Chemotherapy has been studied, but its role in adjuvant therapy for ependymomas is not established, although it may be considered in certain cases, such as residual disease or salvage therapy 4, 5
• The latest classification of ependymoma by the World Health Organization (WHO) takes into account molecular information, which may lead to more complex treatment strategies 5