Brain and other CNS tumors account for 1-2% of all cancers – RARE CANCER

1. Relative survival after diagnosis with a primary brain or other CNS tumor in the National Program of Cancer Registries, 2001-2014. Quinn T. Ostrom, Gabrielle Truitt, Haley Gittleman, Carol Kruchko, Reda Wilson, Jill S. Barnholtz-Sloan

2. Brain and other CNS tumors account for 1-2% of all cancers – RARE CANCER Estimated New US Cancer Cases 2017 Brain and other CNS 1.6% Brain and other CNS 1.2% ACS 2017

3. Brain and other CNS tumors are an extremely heterogeneous group of histologies CBTRUS 2018

4. Cancer Survival Statistics • The majority of reported cancer survival statistics in the US are generated using the National Cancer Institute’s publically available Surveillance, Epidemiology, and End Results data • Prior to 2019 represented 28% of the US population (now 34%). • In the case of rare cancers or special subpopulations, datasets based on a larger portion of the US population may allow for analyses focused on: • Rare cancer types with low incidence • Special subpopulations. • Specific brain and other CNS tumor histologies are relatively rare, use of larger datasets allow for more detailed analyses and contribute to new insights in these tumors.

5. Materials and Methods • Survival data from the National Program of Cancer Registries • Covers 81% of US Population • Survival time was calculated by various types of file linkage. • Covered years 2001-2014 for malignant tumors and 2004-2014 for non-malignant tumors • Histologies were categories using the CBTRUS histologic classification scheme. • Relative survival rates were generated by histology and behavior overall, and by sex, age, and race/ethnicity using SEER*Stat

6. Source of Survival Follow-Up

7. Overall Survival by Behavior • The final survival dataset included 224,609 malignant tumors and 314,614 nonmalignant tumors • Five-year relative survival: • 35.4% for malignant tumors • 89.8% for non-malignant tumors.

8. Survival by Malignant Histologies • Glioblastoma was the malignant histology with the poorest five- and ten-year survival • Pilocytic astrocytoma had the highest survival, followed by ependymal tumors

9. Survival by Non-Malignant Histologies • Craniopharyngioma was the non-malignant histology with the poorest survival • Nerve sheath tumors had the highest survival rates

10. Age Differences in Survival • In general, survival decreased with increasing age, with the poorest outcomes in individuals 65+ years old • Children (ages 0-14 years) and Adolescents and Young Adults (ages 15-39 years) had the highest survival in most histologic groups

11. Age Differences in Survival - Pediatric • Within 5 years for pediatric age-groups, survival patterns varied significantly by histology • In glioblastoma, the youngest age- group had the highest survival outcomes • In other histologies, this youngest age-group had the poorest survival

12. Age Differences in Survival - Adults • Within 10 years for adult age- groups, survival outcomes were decreased with increasing age • In most histologies, those 20-44 years old had the highest overall survival • Individuals age 75+ years had the poorest survival outcomes

13. Sex Differences in Survival • Survival in both malignant and non-malignant tumors was highest in females as compared to males • This was true for most histologies, with the exception of germ cell tumors, where survival was highest in males

14. Racial/Ethnic Differences in Survival • Overall survival after diagnosis with malignant and non-malignant tumors was highest in white Hispanics . • This pattern was also observed in most specific histologies, while White non-Hispanics and Blacks had the poorest survival outcomes. • Survival among white Hispanics may be overestimated due to difficulty in obtaining accurate survival information if individuals leave the country.

15. Conclusions • Survival after diagnosis with primary brain tumor varied by behavior, histology, sex, race/ethnicity and age at diagnosis. • Younger age and female sex were associated with increased survival for many histologies. • The worst five-year survival outcomes were in glioblastoma, while non-malignant nerve-sheath tumors had the best survival outcomes. • Differences in relative survival may be due not only to ‘true’ differences in outcomes between groups, but ability to obtain accurate follow up information may vary between groups. • These survival outcomes are comparable to those estimations using other datasets and likely accurately represent national survival patterns.

16. Future Directions • Beginning with collection year 2018, US registries are now collecting selected molecular markers for brain and other CNS tumors, which will be available in analysis year 2021. • These markers are strong prognostic factors in many subtypes, and are essential to clinically relevant brain and other CNS tumor survival statistics. • Starting with its 2019 annual statistical report, CBTRUS will now use these data when generating survival statistics

17. Acknowledgments and Funding CBTRUS Analytic Group: Carol Kruchko, Jill Barnholtz-Sloan, Gabrielle Truitt, Haley Gittleman, Gino Cioffi, Nirav Patil Funding for CBTRUS was provided by: • Centers for Disease Control and Prevention (CDC) under Contract No. 2016-M-9030 • National Cancer Institute (NCI) under Contract No. HHSN261201800176P • American Brain Tumor Association • The Sontag Foundation • Novocure • AbbVie • The Musella Foundation • National Brain Tumor Society • The Zelda Tetenbaum Memorial Fund • The Uncle Kory Foundation • Private and in kind donations. Contents are solely the responsibility of the authors and do not necessarily reflect the official views of the CDC nor of the NCI. QTO was funded by a Research Training Grant from the Cancer Prevention and Research Institute of Texas (CPRIT; RP160097T).

18. Questions?