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Descriptive epidemiology of ependymal tumours in the United States
J L Villano1, C K Parker, T A Dolecek
1Departments of Medicine and Neurology, Division of Oncology, University of Kentucky, Lexington, KY, USA. jlvillano@uky.edu
Insights
Ependymomas impact children and adults, but pediatric patients face worse survival rates due to higher rates of aggressive anaplastic ependymomas and intracranial disease.
Area of Science:
- Neuro-oncology
- Epidemiology
- Pediatric Oncology
Background:
- Ependymomas are rare primary gliomas affecting both children and adults.
- Survival rates for pediatric ependymoma patients are notably worse than for adults.
Purpose of the Study:
- To evaluate the incidence and survival rates of ependymoma.
- To identify factors contributing to survival disparities between pediatric and adult ependymoma patients.
Main Methods:
- Utilized data from the Central Brain Tumor Registry of the United States and the SEER 13 registries.
- Analyzed incidence and survival data for brain and central nervous system ependymal tumors.
- Examined tumor site, histology, and age-adjusted incidence rates.
Main Results:
- The incidence rate of ependymomas was 0.41/100,000.
- Spinal cord tumors were more common in adults (50-60%), while brain tumors were more prevalent in children (approx. 80%).
- Anaplastic ependymomas were more frequent in children (30%) than adults (3-5%), correlating with lower survival rates.
Conclusions:
- Pediatric ependymoma cases exhibit worse outcomes compared to adults.
- Higher percentages of anaplastic ependymomas and intracranial disease contribute to poorer pediatric survival.
- Understanding these disparities is crucial for improving pediatric ependymoma treatment and outcomes.
Background:
Ependymomas are rare primary gliomas that commonly affect both children and adults, but unique as survival is worse in children.
Methods:
Data on brain and central nervous system primary malignant and non-malignant ependymal tumours from the Central Brain Tumor Registry of the United States analytic data set and primary malignant ependymal tumours from the SEER 13 registries research data file were used to evaluate incidence and survival, respectively.
Results:
The 2004-2009 average annual age-adjusted incidence rate of ependymal tumours was 0.41/100,000. Spinal cord/cauda equina was the primary site at diagnosis for 50-60% of ependymal tumours in adult age groups in contrast to about 20% in children and adolescents. Ependymoma was the most frequent histology in all age groups; however, anaplastic ependymoma comprised about 30% in cases 0-19 years of age compared with about 3-5% in adult age groups. Overall, relative survival was favourable with rates at ∼85% and 75% at 3 and 10 years post diagnosis, respectively. However, children and adolescents, the oldest adult age group, cases diagnosed with anaplastic ependymoma and/or tumour location in a brain site had lowest survival rates.
Conclusion:
Paediatric cases had worse outcomes compared with adults for numerous reasons including having a higher percentage of anaplastic ependymomas and greater percentage of cases of intracranial disease.
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