CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2017-2021

Abstract

The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population. This report contains the most up-to-date population-based data on primary brain tumors available and supersedes all previous reports in terms of completeness and accuracy. All rates are age-adjusted using the 2000 US standard population and presented per 100,000 population. Between 2017 and 2021, the average annual age-adjusted incidence rate (AAAIR) of all primary malignant and non-malignant brain and other CNS tumors was 25.34 per 100,000 population (malignant AAAIR=6.89 and non-malignant AAAIR=18.46). This overall rate was higher in females compared to males (28.77 versus 21.78 per 100,000) and non-Hispanic Black persons compared to persons who were non-Hispanic White (26.60 versus 25.72 per 100,000), non-Hispanic American Indian/Alaska Native (23.48 per 100,000), non-Hispanic Asian or Pacific Islander (19.86 per 100,000), and Hispanic persons of all races (22.37 per 100,000). Gliomas accounted for 22.9% of all tumors. The most commonly occurring malignant brain and other CNS histopathology was glioblastoma (13.9% of all tumors and 51.5% of all malignant tumors), and the most common predominantly non-malignant histopathology was meningioma (41.7% of all tumors and 56.8% of all non-malignant tumors). Glioblastomas were more common in males, and meningiomas were more common in females. In children and adolescents (ages 0-19 years), the incidence rate of all primary brain and other CNS tumors was 6.02 per 100,000 population. There were 87,053 deaths attributed to malignant brain and other CNS tumors between 2017 and 2021. This represents an average annual mortality rate of 4.41 per 100,000 population and an average of 17,411 deaths per year. The five-year relative survival rate following diagnosis of a malignant brain or other CNS tumor was 35.7%. For a non-malignant brain or other CNS tumor the five-year relative survival rate was 92.0%.

Figure 1.

Availability by Central Cancer Registry for SEER and NPCR Incidence Data (2017-2021) and Survival Data (2001-2020)

Figure 2.

Overview of CBTRUS Data Edits Workflow

Figure 3.

A) Annual Age-Adjusted Incidence Rates^a of All Primary Brain and Other Central Nervous System Tumors by Year and Behavior, and B) Average Annual Age-Adjusted Incidence Rates of All Primary Brain and Other Central Nervous System Tumors by Age Group at Diagnosis and Behavior

Figure 4.

Average Annual Age-Adjusted Incidence Rates^a with 95% Confidence Intervals of All Primary Brain and Other Central Nervous System Tumors in Comparison to the Top Eight Highest Incidence Cancers Among A) Children Ages 0-14 Years, C) Adolescents and Young Adults Ages 15-39 Years, and E) Older Adults Ages 40+ Years; and Average Annual Age-Adjusted Mortality with 95% Confidence Intervals of All Primary Brain and Other Central Nervous System Tumors in Comparison to the Top Five Causes of Cancer Death and Top Three Non-Cancer Causes of Death for B) Children Ages 0-14 Years, D) Adolescents and Young Adults Ages 15-39 Years, and F) Older Adults Ages 40+ Years

Figure 5.

Completeness of the Brain Molecular Marker Variable^a and Selected Histopathologies by ICD-O-3 Code by Year of Diagnosis

Figure 6.

Frequency of IDH Mutations^a by WHO Grade for Selected Astrocytoma Histopathologies^b

Figure 7.

Frequency of Molecularly Defined Medulloblastoma Subtypes^a

Figure 8.

Annual Age-Adjusted Incidence Rates^a and Annual Percent Change of Selected Brain and Other Central Nervous System Tumors by A) Behavior, B) Meningiomas and Select Gliomas, and C) Embryonal Tumors

Figure 9.

Distribution^a of Primary Brain and Other Central Nervous System Tumors by Behavior (Five-Year Total=467,894; Annual Average Cases=93,579)

Figure 10.

Distribution^a of All Primary Brain and Other Central Nervous System Tumors (Malignant and Non-Malignant Combined; Five-Year Total=467,894; Annual Average Cases=93,579) by A) Site and B) Histopathology

Figure 11.

Distribution^a of Malignant Primary Brain and Other Central Nervous System Tumors (Five-Year Total=126,807; Annual Average Cases=25,361) by A) Site and B) Histopathology

Figure 12.

Distribution^a of All Non-Malignant Primary Brain and Other Central Nervous System Tumors (Five-Year Total=341,087; Annual Average Cases=68,217) by A) Site and B) Histopathology

Figure 13.

Distribution^a of Primary Brain and Other Central Nervous System Gliomas (ICD-O-3 histopathology codes 9380-9384 and 9391-9460) (Five-Year Total=107,219; Annual Average Cases=21,444) by A) Site and B) Histopathology Subtypes

Figure 14.

Distribution^a of Primary Spinal Cord, Spinal Meninges, and Cauda Equina Tumors by Histopathology in A) Children and Adolescents (Ages 0-19 Years, Five-Year Total=1,368; Annual Average Cases=274) and B) Adults (Ages 20+ Years, Five-Year Total=18,966; Annual Average Cases=3,793)

Figure 15.

Average Annual Age-Adjusted Incidence Rates^a of Brain and Other Central Nervous System Tumors by Selected Histopathologies and Age Group at Diagnosis for A) Children Ages 0-14 Years, B) Adolescents and Young Adults Ages 15-39 Years, and B) Older Adults Ages 40+ Years

Figure 16.

Distribution of Age at Diagnosis (Point at Median) By Selected Primary Brain and Other Central Nervous System Tumor Histopathologies

Figure 17.

Distribution^a in Children and Adolescents (Ages 0-19 Years) of Primary Brain and Other Central Nervous System Tumors (Five-Year Total=24,776; Annual Average Cases=4,954) by A) Site and B) Histopathology

Figure 18.

Distribution^a in Children (Ages 0-14 Years) of Primary Brain and Central Nervous System Tumors (Five-Year Total=16,905; Annual Average Cases=3,381) by A) Site and B) Histopathology

Figure 19.

Distribution^a in Adolescents (Ages 15-19 Years) of Primary Brain and Central Nervous System Tumors (Five-Year Total=7,871; Annual Average Cases=1,574) by A) Site and B) Histopathology

Figure 20.

Distribution^a in Adolescents and Young Adults^b (Ages 15-39 Years) of Primary Brain and Other Central Nervous System Tumors (Five-Year Total=65,399; Annual Average Cases=13,080) by A) Site and B) Histopathology

Figure 21.

Average Annual Age-Adjusted Incidence Rates^a of All A) Malignant and B) Non-Malignant Primary Brain and Other Central Nervous System Tumors by Central Cancer Registry

Figure 22.

Incidence Rate Ratios by Sex (Males:Females) for Selected Primary Brain and Other Central Nervous System Tumor Histopathologies

Figure 23.

Incidence Rate Ratios by Race and Hispanic Ethnicity by A) Non-Hispanic White:Non-Hispanic Black, B) Non-Hispanic White:Non-Hispanic Asian or Pacific Islander, and C) Non-Hispanic White:Hispanic (All Races) for Selected Primary Brain and Other Central Nervous System Tumor Histopathologies

Figure 24.

Average Annual Age-Adjusted Mortality Rates^a for Malignant Primary Brain and Other Central Nervous System Tumors by Central Cancer Registry