Epidemiologic and molecular prognostic review of glioblastoma

Abstract

Glioblastoma multiforme (GBM) is the most common and aggressive primary central nervous system malignancy with a median survival of 15 months. The average incidence rate of GBM is 3.19/100,000 population, and the median age of diagnosis is 64 years. Incidence is higher in men and individuals of white race and non-Hispanic ethnicity. Many genetic and environmental factors have been studied in GBM, but the majority are sporadic, and no risk factor accounting for a large proportion of GBMs has been identified. However, several favorable clinical prognostic factors are identified, including younger age at diagnosis, cerebellar location, high performance status, and maximal tumor resection. GBMs comprise of primary and secondary subtypes, which evolve through different genetic pathways, affect patients at different ages, and have differences in outcomes. We report the current epidemiology of GBM with new data from the Central Brain Tumor Registry of the United States 2006 to 2010 as well as demonstrate and discuss trends in incidence and survival. We also provide a concise review on molecular markers in GBM that have helped distinguish biologically similar subtypes of GBM and have prognostic and predictive value.

Figure 1

Age Adjusted Incidence Rates of Glioblastoma by Region in US, CBTRUS Statistical Report, SEER 2006–2010. Rates are per 100,000 (2). Abbreviations: CBTRUS, Central Brain Tumor Registry of United States.

Figure 2

Age-Adjusted and Age-Specific Incidence Rates for Glioblastoma by Age at Diagnosis and Gender, CBTRUS Statistical Report: NPCR and SEER, 2006–2010. X axis: age groups; Y axis: incidence rates. Rates are per 100,000 and age-adjusted to the 2000 US standard population. Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, CDC's National Program of Cancer Registries; SEER, NCI's Surveillance, Epidemiology and End Results program (2).

Figure 3

3A: Average Annual Age Adjusted Incidence Rates of Glioblastoma by Race, CBTRUS Statistical Report: NPCR and SEER, 2006–2010. X axis: race; Y axis: incidence rates. 3B: Average Annual Age Adjusted Incidence Rates of Glioblastoma by Ethnicity, CBTRUS Statistical Report: NPCR and SEER, 2006–2010. X axis: ethnicity; Y axis: incidence rates. Rates are per 100,000. Abbreviations: IR: Incidence Rate; AIAN, Asian Indian Alaskan Native; API, Asian Pacific Islander; CBTRUS, Central Brain Tumor Registry of United States; NCPR, National Program of Cancer Registries (2).

Figure 4

4A: Relative Survival Rates for Glioblastoma, SEER 18 Registries, 1995–2010. 4B: Relative Survival Rates for Glioblastoma by Gender, SEER 18 registries, 1995–2010. Number of cases in each gender: male, 16,221; female: 11,993. 4C: Relative Survival Rates for Glioblastoma by Race, SEER 18 registries, 1995–2010. Number of cases in each race: whites, 25,360; blacks, 1,547; API, 1,151. *Relative survival rates for American Indian/Alaskan native are excluded due to low numbers. X axis: time in years; Y axis: survival in percentage. Rates are an estimate of the percentage of patients alive at one, two, three, four, five, and ten year, respectively. Estimated by CBTRUS using SEER Program (www.seer.cancer.gov) SEER*Stat Database: Incidence - SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2011 Sub (1973–2009 varying) - Linked To County Attributes - Total U.S., 1969–2010 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistics Branch, released April 2012, based on the November 2011 submission. Abbreviation: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, CDC's National Program of Cancer Registries; SEER, NCI's Surveillance, Epidemiology and End Results program; API: Asian Pacific Islander (2).

Figure 5

Five and Ten-Year Relative Survival Rate for Glioblastoma by Age, SEER 18 Registries, 1995–2010. X axis: age groups; Y axis: survival in percentage. Rates are in percentage (%). Estimated by CBTRUS using SEER Program (www.seer.cancer.gov) SEER*Stat Database: Incidence - SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2011 Sub (1973–2009 varying) - Linked To County Attributes - Total U.S., 1969–2010 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistics Branch, released April 2012, based on the November 2011 submission. Abbreviation: CBTRUS, Central Brain Tumor Registry of the United States; SEER, NCI's Surveillance, Epidemiology and End Results program (2).

Figure 6

Relationship between molecular markers and the different grades of glioma. Red = primary glioblastoma, blue = grades II, III astrocytomas and secondary glioblastoma, purple = grades II and III oligodendrogliomas. Abbreviations: ATRX, alpha thalassemia/mental retardation syndrome X-linked; IDH1/2, isocitrate dehydrogenase enzyme 1/2 mutation; EGFR, epidermal growth factor receptor, hTERT, high frequency of telomerase reverse transcriptase (hTERT) promoter mutation.