Incidence of primary brain tumors

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Official reprint from UpToDate

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Incidence of primary brain tumors

Authors
Dominique Michaud, Sc.D
David Schiff, MD
Tracy Batchelor, MD, MPH

Section Editors
Jay S Loeffler, MD
Patrick Y Wen, MD

Deputy Editor
April F Eichler, MD, MPH

Disclosures: Dominique Michaud, Sc.D Nothing to disclose. David Schiff, MD Grant/Research/Clinical Trial Support:
AngioChem, Stemline Therapeutics, Cortice Biosciences [Glioblastoma]. Consultant/Advisory Boards: Cavion, Genentech,
Heron Therapeutics, Midatech Pharma, Vascular Biogenics, Celldex Therapeutics, Sigma Tau Pharmaceuticals

All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Oct 2015. | This topic last updated: Nov 11, 2015.
INTRODUCTION Primary brain tumors are a diverse group of neoplasms arising from different cells
of the central nervous system (CNS). Although uncommon, there is evidence that the incidence of these
tumors has been rising for as much as fifty years. (See "Classification of gliomas", section on
'Histopathologic and molecular classification'.)
The incidence of primary malignant and benign brain tumors will be reviewed here. Risk factors for the
development of brain tumors as well as the incidence of brain metastases are discussed separately.
(See "Risk factors for brain tumors" and "Overview of the clinical manifestations, diagnosis, and
management of patients with brain metastases".)
SOURCES OF DATA The Central Brain Tumor Registry of the United States (CBTRUS) includes both
benign and malignant lesions in its data collection. The CBTRUS was established in 1992 and compiles
information from twelve state cancer registries [1]. Earlier data from the SEER program and other US
cancer registries were limited to malignant brain tumors; however, as of 2004, data on nonmalignant
brain tumors have been systematically collected by US cancer registries after the passage of Public Law
107-260, the Benign Brain Tumor Cancer Registries Amendment Act [2].
Inclusion of benign lesions is important to properly evaluate differences in etiology and to assess the
potential impact of intervention studies. Benign lesions often produce the same neurologic symptoms as
malignant tumors and distinguishing between benign and malignant based upon clinical grounds can be
difficult. In addition, both benign and malignant lesions are frequently treated similarly with either surgical
intervention or radiation therapy, and many malignant brain tumors probably originate from benign
precursors. (See "Classification of gliomas", section on 'Histopathologic and molecular classification'.)
INCIDENCE AND MORTALITY The US incidence rate for primary brain and nervous system tumors
in adults (aged 20 years or older) is estimated to be 28.6 per 100,000 persons (data from 51 cancer
registries, 2008 to 2012) [3]. Approximately one-third of tumors are malignant and the remainder are
benign or borderline malignant [2,3].
The incidence rate for children (aged 0 to 19 years) is much lower (5.6 per 100,000 children) [3],
although a higher percentage of primary brain tumors are malignant in children compared with adults (65
versus 33 percent) [2].
Mortality Although brain tumors account for only 2 percent of all cancers, these neoplasms result in a
disproportionate share of cancer morbidity and mortality. The annual age-adjusted mortality rate for
primary malignant brain and CNS tumors in the United States (2008 to 2012) was 5.8 per 100,000
population [4]. Including all ages and all races, the approximate five-year survival rate for malignant brain

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Incidence of primary brain tumors

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tumors is 34 percent [3,5]. The five-year survival rates for the most common histologic subtypes,
anaplastic astrocytoma and glioblastoma, are 28 percent and 5 percent, respectively [3].
For all histologic types, pediatric and young adult populations have a better survival than do older adults.
As an example, for all primary malignant brain tumors combined, the five year survival rate among
children under age 14 is 62 percent, compared with 5 percent in adults 65 years of age and older [5].
Overall survival in patients with malignant brain tumors has not improved significantly over the last fifty
years. However, survival rates vary according to age and histologic type. As an example, there were
modest gains in survival between 1975 and 1995 for those younger than 65, but not older individuals [6].
Although little progress has been made in survival from glioblastoma in the last thirty years, five-year
survival rates for medulloblastoma increased by 20 percent from the 1970s to the 1980s, although data
suggest a leveling off of survival rates [7].
In general, young age, high performance status, and lower pathologic grade are favorable prognostic
factors for primary brain tumors. Less significant predictors of favorable prognosis include long duration
of symptoms, absence of mental changes at time of diagnosis, cerebellar location of tumor, small
preoperative tumor size, and completeness of surgical resection [8].
Gender There is a slight male predominance in the incidence of malignant brain tumors. However,
when both malignant and benign tumor types are examined, males account for less than one-half of all
cases (42 versus 58 percent) [3]. This difference is primarily explained by the higher incidence of
meningiomas in women (10.6 versus 5.7 per 100,000 person-years) [3].
Race Whites have a higher incidence of primary brain tumors compared with blacks across most
histologies [3,5]. Exceptions include meningioma, tumors of the pituitary, and craniopharyngioma, where
rates for blacks significantly exceed those observed in whites [3]. In general, malignant brain tumors are
less common among Asian/Pacific Islanders and American Indian/Alaskan Natives [3,9], while the
incidence of malignant brain tumors in Hispanics is intermediate between that in whites or African
Americans [3,10].
Age The incidence of brain tumors varies with age and histology (figure 1). Data compiled from
several tumor registries suggest a peak incidence of all primary brain tumors around age 50, although
autopsy series suggest that incidence rises continuously with increasing age [11]. Similarly, data from
CBTRUS indicate that the incidence of the most common primary malignant brain tumor in adults,
glioblastoma, increases with increasing age and is highest in 75 to 84 year olds [3]. Most intracranial
tumors occur in people older than 45 years of age. Glioblastoma rarely occurs before the age of 15 but
dramatically increases after the age of 45. In contrast, medulloblastoma and other embryonal tumors are
uncommon after the age of 20 [12].
Adults In adults, gliomas (principally astrocytoma) account for approximately 30 percent and
meningioma 35 to 40 percent of symptomatic primary brain tumors (table 1). The incidence of
meningioma is likely higher when neuroimaging studies are included because of the large number of
asymptomatic tumors [11]. Pituitary tumors account for 15 to 20 percent, primary central nervous system
lymphoma for 2 to 3 percent, and craniopharyngioma for 1 percent.
Children In children and adolescents up to the age of 19 years, brain tumors are the most
common solid malignancy and the second leading cause of cancer death after leukemia. Brain tumor
incidence rates are approximately 5.8 per 100,000 per year and the male-to-female ratio is
approximately equal [3].
The different histologic subtypes of neuroepithelial brain tumors seen in children and their relative
frequency are shown in the following table (table 2). The most common include low-grade astrocytoma,
medulloblastoma, and high-grade glioma (anaplastic gliomas and glioblastoma).

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Temporal trends Multiple studies have documented rising incidence rates for brain tumors in several
industrialized countries; this increase seems to be confined mainly to the elderly population, with no
clear ethnic, gender or geographic differences. The incidence in whites rose 80 percent from 1950 to
1994 and 18 percent from 1973 to 1994 [5,13,14].
The increases observed in the United States were present in all age strata and racial groups, similar to
findings in other parts of the world [15]. There is limited epidemiological evidence from a longitudinally
studied population in Rochester, Minnesota suggesting that a rise in brain tumor mortality may have
been evident since the 1940s [16].
The precise etiology of this increase in brain tumor incidence remains unclear. There is agreement that
at least part of this increase is the result of more complete case ascertainment with improved diagnostic
technology [11,17].
However, improved diagnostic capability cannot fully account for the magnitude of the observed increase
in brain tumor incidence [9,17]. These data, in conjunction with the evidence suggesting the increase
may have been occurring for many decades, leave open the possibility that an environmental exposure
may account for part of the increasing incidence of brain tumors. (See "Risk factors for brain tumors".)
International trends and migrant studies Increasing incidence rates of brain tumors have been
observed in many other countries of the world over the last thirty years. The highest incidence rates are
noted in industrialized nations such as the United States, Canada, Australia, and the United Kingdom,
while developing nations have lower rates. In general, the brain cancer incidence appears to be
associated with the level of economic development; differences in the availability of diagnostic
methodology (CT, MRI, neurosurgical technology) may account for some of the observed disparities.
Despite sparse data from developing countries, it appears that most migrants acquire the brain cancer
incidence rates of their host country. For most migrant studies, the host country had elevated incidence
rates compared to the country of origin, with migrants developing increases in brain cancer incidence
and mortality. This observation suggests that environmental factors may influence the development of
brain cancer. However, these studies were complicated by disparities in case ascertainment in the
countries under study.
Primary central nervous system lymphoma There was an overall increase in the incidence of
primary central nervous system lymphoma from the 1960s to the 1990s, which has been attributed at
least in part to an increased frequency of these tumors in immunocompromised populations. (See
"Clinical presentation, pathologic features, and diagnosis of primary central nervous system lymphoma",
section on 'Epidemiology'.)
SUMMARY
Benign tumors can produce substantial morbidity and can occasionally be fatal. As of 2004, data on the
benign tumors are being collected systematically by all cancer registries. (See 'Sources of data' above.)
The most common malignant brain tumors, glioblastomas, are rapidly fatal.
Mortality data on individuals with brain tumors indicate that younger patients have a significantly
better prognosis compared to older individuals. (See 'Mortality' above.)
The incidence of brain tumors varies with age and histology. Gliomas and meningiomas are most
common tumors in adults, while there are a number of less common tumor types that are seen
almost exclusively in children (table 2). (See 'Age' above.)
The overall incidence of brain tumors has been rising. This is most likely due to more frequent
diagnosis with improved imaging modalities, but there is a possibility that environmental factors
may be contributing to a true increase in incidence as well. (See 'Temporal trends' above.)

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REFERENCES
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