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Background: Ependymoma is a histologically defined central nervous system tumor most commonly occurring in childhood. Population-level incidence differences by race/ethnicity are observed, with individuals of European ancestry at highest risk. We aimed to determine whether extent of European genetic ancestry is associated with ependymoma risk in US populations., Methods: In a multi-ethnic study of Californian children (327 cases, 1970 controls), we estimated the proportions of European, African, and Native American ancestry among recently admixed Hispanic and African American subjects and estimated European admixture among non-Hispanic white subjects using genome-wide data. We tested whether genome-wide ancestry differences were associated with ependymoma risk and performed admixture mapping to identify associations with local ancestry. We also evaluated race/ethnicity-stratified ependymoma incidence data from the Central Brain Tumor Registry of the United States (CBTRUS)., Results: CBTRUS data revealed that African American and Native American children have 33% and 36%, respectively, reduced incidence of ependymoma compared with non-Hispanic whites. In genetic analyses, a 20% increase in European ancestry was associated with a 1.31-fold higher odds of ependymoma among self-reported Hispanics and African Americans (95% CI: 1.08-1.59, Pmeta = 6.7 × 10-3). Additionally, eastern European ancestral substructure was associated with increased ependymoma risk in non-Hispanic whites (P = 0.030) and in Hispanics (P = 0.043). Admixture mapping revealed a peak at 20p13 associated with increased local European ancestry, and targeted fine-mapping identified a lead variant at rs6039499 near RSPO4 (odds ratio = 1.99; 95% CI: 1.45-2.73; P = 2.2 × 10-5) but which was not validated in an independent set of posterior fossa type A patients., Conclusions: Interethnic differences in ependymoma risk are recapitulated in the genomic ancestry of ependymoma patients, implicating regions to target in future association studies., (© The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Ependymomas are rare CNS tumors. Previous studies describing the clinical course of ependymoma patients were restricted to small sample sizes, often with patients at a specific institution., Methods: Clinically annotated ependymoma tissue samples from 19 institutions were centrally reviewed. Patients were all adults aged 18 years or older at the time of diagnosis. Potential prognostic clinical factors identified on univariate analysis were included in a multivariate Cox proportional hazards model with backwards selection to model progression-free survival., Results: The 282 adult ependymoma patients were equally male and female with a mean age of 43 years (range, 18-80y) at diagnosis. The majority were grade II (78%) with the tumor grade for 20 cases being reclassified on central review (half to higher grade). Tumor locations were spine (46%), infratentorial (35%), and supratentorial (19%). Tumor recurrence occurred in 26% (n = 74) of patients with a median time to progression of 14 years. A multivariate Cox proportional hazards model identified supratentorial location (P < .01), grade III (anaplastic; P < .01), and subtotal resection, followed or not by radiation (P < .01), as significantly increasing risk of early progression., Conclusions: We report findings from an ongoing, multicenter collaboration from a collection of clinically annotated adult ependymoma tumor samples demonstrating distinct predictors of progression-free survival. This unique resource provides the opportunity to better define the clinical course of ependymoma for clinical and translational studies., (© The Author(s) 2014. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

The etiology of primary brain tumors is largely unknown. Since a peak of incidence occurs during childhood, factors operating very early in life might play a key role. Previous studies have suggested that high birth weight is associated with an increased brain tumor risk. The authors conducted a meta-analysis on the association between birth weight and risk of specific histologic types of primary brain tumors. They included published studies (1966-2007) that reported odds ratios and 95% confidence intervals for brain tumors associated with birth weight. The authors identified eight studies involving 1,748,964 children, of whom 4,162 suffered from brain tumors of three histologic types (astrocytoma, medulloblastoma, and ependymoma). For astrocytoma, high birth weight (>4,000 g) was associated with increased risk (odds ratio = 1.38, 95% confidence interval (CI): 1.07, 1.79), with each 1,000-g increase in birth weight being associated with a 19% (95% CI: 4, 36) increase in risk. For medulloblastoma, high birth weight was also positively associated with increased risk (odds ratio = 1.27, 95% CI: 1.02, 1.60). No association was found for ependymoma. These findings indicate that birth weight is related to the development of childhood brain tumors, with high birth weight being a risk factor for the two most common types of brain tumors.

Incidence rates for CNS tumours in children of age 0-14 years in the Region of Lombardy, Italy, during the period 1988-93 were analysed; survival probability updated to December 1995 was also estimated. CNS tumours defined according to the International Classification of Diseases for Oncology codes were actively searched for. CNS tumours were diagnosed in 296 children. The age-standardized rates were 40.0 per million child years for both sexes together, and 45.3 for boys and 34.4 for girls. In all age groups, boys had a higher incidence than girls. The annual incidences were 13.7, 7.0, 5.8 for astrocytoma, medulloblastoma and ependymoma, respectively. The overall survival percentages at 5 and 8 years after diagnosis were 68 and 66, respectively. Prognosis was good for astrocytoma (5-year survival, 81%), and declined in the order: other gliomas (5-year survival, 76%); ependymoma (5-year survival, 62%), and medulloblastoma (5-year survival, 43%). The histological type of the tumour was the most powerful independent predictor of survival in children with a CNS tumour. Medulloblastoma/primitive neuroectodermal tumours appeared to have the highest risk of a poor prognosis compared with astrocytoma (relative risk, 3.27; 95% confidence interval, 1.81-5.91). Age at diagnosis and sex had no significant effect on survival. The incidence of childhood CNS tumours found in this study is higher than previously reported in Italy, and is one of the highest in the world from population-based data. Survival of children with brain tumours has improved greatly in recent years. These results suggest that children in Lombardy with CNS tumours had a good survival experience compatible with high quality of care.

The age-specific incidence pattern for tumors of the brain and cranial meninges in Connecticut over a 30-year period shows an early peak followed by a taller and sharper peak with a maximum in the 55-65 year age group. This overall curve reflects the pattern shown for glioblastoma, the tumor accounting for the majority of the histologically confirmed cases. The reported rates are probably underestimates of the actual rates. The various histologic types of brain tumors reveal sufficiently distinct epidemiologic patterns to be considered as separate diseases.

Data from nine population-based cancer registries from the National Cancer Institute's Surveillance, Epidemiology, and End Results program of the United States were used to study the incidence of individual histologic types of malignant central nervous system tumors by age and sex among adults. On a log-log scale, incidence increased linearly between the ages 35 and 64, with a slope that was not different between males and females or among registries but that varied by histologic type. The estimated slopes were 0.4 for ependymomas, 1.0 for oligodendrogliomas, 1.7 for astrocytomas, 2.8 for meningiomas, and 3.9 for glioblastomas. The rate at which incidence increased with age was significantly higher for glioblastomas than for other glial tumors. This finding suggests a different mechanism of carcinogenesis for glioblastomas than for other glial tumors.