Ependymomas are slow-growing central nervous system tumors that involve the ventricular system. Diagnosis is based on MRI and biopsy. Treatment is a combination of surgery, radiation therapy, and sometimes chemotherapy.
Ependymomas are the third most common central nervous system tumor in children (after astrocytomas and medulloblastomas), representing 10% of pediatric brain tumors (1). Mean age at diagnosis is 6 years; however, about 30% of ependymomas occur in children < 3 years old.
Ependymomas are derived from the ependymal lining of the ventricular system and can occur in the supratentorial area, posterior fossa, or the spine. Ependymomas are usually malignant.
In children, ependymomas most commonly occur in the posterior fossa, followed by the supratentorial area, and then least commonly the spine (up to 10% of cases). In contrast, the majority (> 60%) of ependymomas in adults occur in the spine (2).
References
1. Ostrom QT, Price M, Neff C, et al: CBTRUS statistical report: Primary brain and other central nervous system tumors diagnosed in the United States in 2015-2019. Neuro Oncol 24(Suppl 5):v1–v95, 2022. doi: 10.1093/neuonc/noac202
2. McGuire CS, Sainani KL, Fisher PG: Incidence patterns for ependymoma: A surveillance, epidemiology, and end results study. J Neurosurg 110(4):725–729, 2009. doi: 10.3171/2008.9.JNS08117
Symptoms and Signs of Ependymomas
Initial presentation depends on the location of the tumor.
For supratentorial ependymomas, symptoms can include changes in personality or concentration, headaches, seizures, and focal neurologic deficits.
For posterior fossa tumors, symptoms are typically related to increased intracranial pressure. Infants may present with developmental delay, irritability, and increased head circumference. Older children typically present with nausea, vomiting, headache, ataxia/balance issues, and lethargy.
For spinal tumors, symptoms of spinal cord compression (eg, back pain, loss of bladder and bowel control) may occur.
Diagnosis of Ependymomas
MRI
Histologic evaluation of biopsy specimen or entire resected tumor
Diagnosis of ependymoma is based on MRI.
Definitive diagnosis is made using tumor tissue obtained by biopsy or ideally by gross total resection of the tumor at initial presentation.
(See also the World Health Organization's 2021 classification of tumors of the central nervous system.)
Treatment of Ependymomas
Surgical resection, usually followed by radiation therapy
Sometimes chemotherapy
Surgical resection is critical, and the degree of resection is one of the most important prognostic factors.
Radiation therapy has been shown to increase survival and should be given after surgery; however, a small subset of patients with supratentorial ependymomas who do not receive radiation can potentially can be cured by surgery alone.
Chemotherapy has not been clearly shown to improve survival but, in some children, may be used to shrink the tumor before gross total resection or a second-look surgery.
Prognosis for Ependymomas
Survival rate depends on age and on how much of the tumor can be removed:
Complete or near-complete removal: 51 to 80% survival (1)
Less than 90% removal: 0 to 26% survival (1)
Children who survive are at risk of neurologic deficits resulting from the adverse effects of surgery and radiation therapy.
Prognosis reference
1. Cage TA, Clark AJ, Aranda D, et al: A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr 11(6):673–681, 2013. doi: 10.3171/2013.2.PEDS12345
More Information
The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
World Health Organization (WHO): The 2021 WHO Classification of Tumors of the Central Nervous System: A summary
