Descriptive epidemiology of ependymal tumours in the United States.

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.