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. 2021 Jun 1;23(6):990-998.
doi: 10.1093/neuonc/noaa295.

Epidemiology of brainstem high-grade gliomas in children and adolescents in the United States, 2000-2017

Nirav Patil  1 , Michael E Kelly  2 , Debra Nana Yeboa  3 , Robin A Buerki  4 , Gino Cioffi  1   5 , Sweta Balaji  6 , Quinn T Ostrom  1   7 , Carol Kruchko  1 , Jill S Barnholtz-Sloan  1   5   8
Affiliations

Epidemiology of brainstem high-grade gliomas in children and adolescents in the United States, 2000-2017

Nirav Patil et al. Neuro Oncol. .

Abstract

Background: Limited population-based data exist for the brainstem gliomas for children ages ≤19 years, which includes high-grade aggressively growing tumors such as diffuse intrinsic pontine glioma (DIPG). We examined the overall incidence and survival patterns in children with brainstem high-grade glioma (HGG) by age, sex, and race and ethnicity.

Methods: We used data from Central Brain Tumor Registry of the United States (CBTRUS), obtained through data use agreements with the Centers for Disease Control (CDC) and the National Cancer Institute (NCI) from 2000 to 2017, and survival data from the CDCs National Program of Cancer Registries (NPCR), from 2001 to 2016 for malignant brainstem HGG for ages ≤19 years (per WHO ICD-O-3 codes). HGG was determined by established histologic and/or imaging criteria. Age-adjusted incidence rates and survival data were used to assess differences overall and by age, sex race, and ethnicity.

Results: The incidence of brainstem HGG was higher among the female and Non-Hispanic population. Majority (69.8%) of these tumors were diagnosed radiographically. Incidence was higher in children aged 1-9 years compared to older children. Whites had a higher incidence compared to Blacks. However, the risk of death was higher among Blacks and Other race compared to Whites. There was no difference in survival by sex.

Conclusions: We report the most comprehensive incidence and survival data on these lethal brainstem HGGs. Incidence and survival among patients with brainstem HGGs differed significantly by race, ethnicity, age-groups, and grade.

Keywords: CBTRUS; DIPG; brainstem; glioma; high-grade glioma.

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Figures

Fig. 1.
Fig. 1.
Distribution of malignant primary childhood and adolescent (age ≤19 years) brain and other CNS high grade gliomas by (A) site (N = 14,402) and B) for brainstem (ICD-O-3 site code C71.7) only by grade (N = 4,486); 2000-2017, CBTRUS.
Fig. 2
Fig. 2
Kaplan-Meier survival curve for primary brainstem high-grade glioma in children and adolescents (age ≤19 years) by (A) sex (B) race (White, Blacks vs Others) (C) age group (<1 year, 01-04 year, 05-09 years, 10-14 years and 15-19 years) (D) ethnicity (Hispanic vs Non-Hispanic) and (E) grade; NPCR 2001-2016 (N = 3,665).
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References

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