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Benign tumors can result in similar symptoms and prognoses as malignant tumors. For
this reason, many cancer registries have routinely included both benign and malignant
intracranial tumors. The U.S. Surveillance, Epidemiology, and End Results (SEER)
network of population-based cancer registries now reports both benign and malignant
CNS tumors. Brain tumor patients may present with general symptoms, such as
headaches and seizures, which result from an increase in intracranial pressure. One-
third of patients present with headaches and one-fifth with seizures. Other symptoms
may include specific motor, speech, or sensory deficits resulting from compression of
the corresponding region of the brain; slow-growing tumors may present with change in
character only.

The discussion in this research paper will include benign and malignant tumors of the
brain, cranial nerves, and cranial meninges, which account for 95% of all CNS tumors
and 93% of all nervous system tumors. For simplicity, this group of tumors will be called
brain tumors or, when benign tumors are excluded, brain cancer. The term central
nervous system tumors (or cancer) indicates that tumors of the spinal cord and spinal
meninges are included along with brain tumors, and nervous system (NS) tumors
indicates that tumors of the peripheral nerves are included as well. Three major
histologic groups are used in the descriptive tables of this research paper,
corresponding to International Classification of Diseases for Oncology (ICD-O-3)
morphology codes 9530–9534 and 9537–9539 for gliomas, 9530–9534 and 9537–
9539 for meningiomas, and 9540–9571 for nerve sheath tumors. The statistics reported
on incidence will focus on data from Surveillance, Epidemiology, and End Results
(SEER).

CLASSIFICATION
ANATOMIC CLASSIFICATION
Tumors of the central nervous system include tumors of the brain, cranial nerves,
cerebral meninges, spinal cord, and spinal meninges. These subsites are represented by
the International Classification of Diseases for Oncology (ICD-O) codes C70.0–C72.9.
We will not include tumors of sites such as the eye and the pituitary gland, which appear
to be etiologically distinct.

HISTOPATHOLOGY
The WHO classification of tumors system has allowed for international standardization
of CNS malignancies based on cell of origin and histopathologic features, including
degree of anaplasia. Tumors are classified into one of four grades: grade 1 is benign,
grade 2 is considered low grade, while grades 3 and 4 are considered high grade or
malignant. The most common are tumors of neuroepithelial origin, including
astrocytomas, oligodendrogliomas, mixed gliomas, ependymal tumors, choroid plexus
tumors, glial tumors of uncertain origin, neuronal and mixed neuronalglial tumors,
neuroblastic tumors, pineal parenchymal tumors, and embryonal tumors. The majority
of CNS primary tumors are of astroglial origin, called gliomas. In the United States,
according to recent data from the Los Angeles County Cancer Surveillance Program,
gliomas account for 55% and 40% of primary CNS tumors among men and women,
respectively (Table 1). The astrocytic tumors account for 80% of gliomas and include
astrocytomas (grade II), anaplastic astrocytomas (grade III), and glioblastoma
multiforme (grade IV). These represent increasing grades of anaplasia and clinical
virulence of tumors of astrocytic cells. Two clinical variants of glioblastoma multiforme
(GBM), primary and secondary, have been described, with primary GBM representing the
more common (75%) of the two forms. Primary GBM is believed to develop de novo and
grows very aggressively without evidence of a malignant precursor lesion. Secondary
GBM is believed to arise from a low-grade lesion that has undergone genetic alterations
to transition to a high-grade tumor. Oligodendrogliomas are classified as tumors with a
cellular morphology most closely resembling that of the normal oligodendrocyte. There
are two grades of oligodendrogliomas in the WHO classification system, where grade II
is the lowest grade of oligodendroglioma and grade III (anaplastic) is the highest.