Brain Tumors

Updated June 2012

Types of Brain Tumors
A brain tumor, known as an intracranial tumor, is an abnormal mass of tissue in which cells
grow and multiply uncontrollably, seemingly unchecked by the mechanisms that control
normal cells. More than 150 different brain tumors have been documented, but the two main
groups of brain tumors are termed primary and metastatic.
Primary brain tumors include tumors that originate from the tissues of the brain, or the brain's
immediate surroundings. Primary tumors are categorized as glial (composed of glial cells) or
non-glial (developed on or in the structures of the brain, including nerves, blood vessels and
glands) and benign or malignant.
Metastatic brain tumors include tumors that arise elsewhere in the body (such as the breast or
lungs) and migrate to the brain, usually through the bloodstream. Metastatic tumors are
considered cancer and are malignant.
Metastatic tumors to the brain affect nearly one in four patients with cancer, or an estimated
150,000 people a year. Up to 40 percent of people with lung cancer will develop metastatic
brain tumors. In the past, the outcome for patients diagnosed with these tumors was very
poor, with typical survival rates of just several weeks. More sophisticated diagnostic tools, in
addition to innovative surgical and radiation approaches, have helped survival rates expand
up to years; and also allowed for an improved quality of life for patients following diagnosis.
Incidence in Adults
The National Cancer Institute estimates that 22,910 adults (12,630 men and 10,280 women)
will be diagnosed with brain and other nervous system tumors in 2012. It also estimates that
in 2012, 13,700 of these diagnoses will result in death.
Between 2005 and 2009, the median age for death from cancer of the brain and other areas of
the nervous system was age 64.
Types of Benign Brain Tumors
Chordomas are benign, slow-growing tumors that are most prevalent in people ages 50 to
60. Their most common locations are the base of the skull and the lower portion of the spine.
Although these tumors are benign, they may invade the adjacent bone and put pressure on
nearby neural tissue. These are rare tumors, contributing to only 0.2 percent of all primary
brain tumors.
Craniopharyngiomas typically are benign, but are difficult tumors to remove because of
their location near critical structures deep in the brain. They usually arise from a portion of
the pituitary gland (the structure that regulates many hormones in the body), so nearly all
patients will require some hormone replacement therapy.

Gangliocytomas, gangliomas and anaplastic gangliogliomas are rare tumors that include
neoplastic nerve cells that are relatively well-differentiated, occurring primarily in young
adults.
Glomus jugulare tumors most frequently are benign and typically are located just under the
skull base, at the top of the jugular vein. They are the most common form of glomus tumor.
However, glomus tumors, in general, contribute to only 0.6 percent of neoplasms of the head
and neck.
Meningiomas are the most common benign intracranial tumors, comprising 10 to 15 percent
of all brain neoplasms, although a very small percentage are malignant. These tumors
originate from the meninges, the membrane-like structures that surround the brain and spinal
cord.
Pineocytomas are generally benign lesions that arise from the pineal cells, occurring
predominantly in adults. They are most often well-defined, noninvasive, homogeneous and
slow-growing.
Pituitary adenomas are the most common intracranial tumors after gliomas, meningiomas
and schwannomas. The large majority of pituitary adenomas are benign and fairly slowgrowing. Even malignant pituitary tumors rarely spread to other parts of the body. Adenomas
are by far the most common disease affecting the pituitary. They commonly affect people in
their 30s or 40s, although they are diagnosed in children, as well. Most of these tumors can be
treated successfully.
Schwannomas are common benign brain tumors in adults. They arise along nerves,
comprised of cells that normally provide the "electrical insulation" for the nerve cells.
Schwannomas often displace the remainder of the normal nerve instead of invading it.
Acoustic neuromas are the most common schwannoma, arising from the eighth cranial nerve,
or vestibularcochlear nerve, which travels from the brain to the ear. Although these tumors
are benign, they can cause serious complications and even death if they grow and exert
pressure on nerves and eventually on the brain. Other locations include the spine and, more
rarely, along nerves that go to the limbs.
Types of Malignant Brain Tumors
Gliomas are the most prevalent type of adult brain tumor, accounting for 78 percent of
malignant brain tumors. They arise from the supporting cells of the brain, called the glia.
These cells are subdivided into astrocytes, ependymal cells and oligodendroglial cells (or
oligos). Glial tumors include the following:

Astrocytomas are the most common glioma, accounting for about half of all primary
brain and spinal cord tumors. Astrocytomas develop from star-shaped glial cells called
astrocytes, part of the supportive tissue of the brain. They may occur in many parts of
the brain, but most commonly in the cerebrum. People of all ages can develop
astrocytomas, but they are more prevalent in adults particularly middle-aged men.
Astrocytomas in the base of the brain are more prevalent in children or younger
people and account for the majority of children's brain tumors. In children, most of
these tumors are considered low-grade, while in adults, most are high-grade.

Ependymomas are derived from a neoplastic transformation of the ependymal cells

lining the ventricular system, and account for two to three percent of all brain tumors.
Most are well-defined, but some are not.

Glioblastoma multiforme (GBM) is the most invasive type of glial tumor. These
tumors tend to grow rapidly, spread to other tissue and have a poor prognosis. They
may be composed of several different kinds of cells, such as astrocytes and
oligodendrocytes. GBM is more common in people ages 50 to 70, and more prevalent
in men than women.

Medulloblastomas usually arise in the cerebellum, most frequently in children. They

are high-grade tumors, but they are usually responsive to radiation and chemotherapy.

Oligodendrogliomas are derived from the cells that make myelin, which is the
insulation for the wiring of the brain.

Other Types of Brain Tumors

Hemangioblastomas are slow-growing tumors, commonly located in the cerebellum. They
originate from blood vessels, can be large in size and often are accompanied by a cyst. These
tumors are most common in people ages 40 to 60 and more prevalent in men than women.
Rhabdoid tumors are rare, highly aggressive tumors that tend to spread throughout the
central nervous system. They often appear in multiple sites in the body, especially in the
kidneys. They are more prevalent in young children, but also can occur in adults.
Pediatric Brain Tumors
Brain tumors in children typically come from different tissues than those affecting adults.
Treatments that are fairly well-tolerated by the adult brain (such as radiation therapy) may
prevent normal development of a child's brain, especially in children younger than age five.
According to the Pediatric Brain Tumor Foundation, approximately 4,200 children are
diagnosed with a brain tumor in the U.S. 72 percent of children diagnosed with a brain tumor
are younger than age 15.
Some types of brain tumors are more common in children than in adults. The most common
types of pediatric tumors are medulloblastomas, low-grade astrocytomas, ependymomas,
craniopharyngiomas and brainstem gliomas.
The World Health Organization (WHO) has developed a grading system to indicate a tumor's
malignancy or benignity based on its histological features under a microscope.

World Health Organization (WHO) Brain Tumor Grades

Grade

Characteristics

Tumor Types

WHO Grade I

Low
Grade

WHO Grade II

WHO Grade
III

High
Grade

WHO Grade IV

Brain Tumor Causes

Least malignant (benign)

Possibly curable via

surgery alone

Pilocytic astrocytoma
Craniopharyngioma
Gangliocytoma
Ganglioglioma

Non-infiltrative

Long-term survival

Slow growing

Relatively slow growing

Somewhat infiltrative

May recur as higher

grade

Malignant

Infiltrative

Tend to recur as higher

grade

Most malignant

Rapid growth, aggressive Gliobastoma multiforme

(GBM)
Pineoblastoma
Widely infiltrative
Medulloblastoma
Ependymoblastoma
Rapid recurrence

Necrosis prone

"Diffuse" Astrocytoma
Pineocytoma
Pure oligodendroglioma

Anaplastic astrocytoma
Anaplastic ependymoma
Anaplastic
oligodendroglioma

Brain tumors are thought to arise when certain genes on the chromosomes of a cell are
damaged and no longer function properly. These genes normally regulate the rate at which the
cell divides (if it divides at all) and repair genes that fix defects of other genes, as well as
genes that should cause the cell to self-destruct if the damage is beyond repair. In some cases,
an individual may be born with partial defects in one or more of these genes. Environmental
factors may then lead to further damage. In other cases, the environmental injury to the genes
may be the only cause. It is not known why some people in an "environment" develop brain
tumors, while others do not.
Once a cell is dividing rapidly and internal mechanisms to check its growth are damaged, the
cell can eventually grow into a tumor. Another line of defense may be the body's immune
system, which optimally would detect the abnormal cell and kill it. Tumors may produce
substances that block the immune system from recognizing the abnormal tumor cells and
eventually overpower all internal and external deterrents to its growth.
A rapidly growing tumor may need more oxygen and nutrients than can be provided by the
local blood supply intended for normal tissue. Tumors can produce substances called
angiogenesis factors that promote the growth of blood vessels. The new vessels that grow
increase the supply of nutrients to the tumor, and, eventually, the tumor becomes dependent
on these new vessels. Research is being done in this area, but more extensive research is
necessary to translate this knowledge into potential therapies.
Symptoms
Symptoms vary depending on the location of the brain tumor, but the following may
accompany different types of brain tumors.

Headaches that may be more severe in the morning

Seizures or convulsions

Difficulty thinking, speaking or articulating

Personality changes

Weakness or paralysis in one part or one side of the body

Loss of balance or dizziness

Vision changes

Hearing changes

Facial numbness or tingling

Nausea or vomiting

Confusion and disorientation

Diagnosis
Sophisticated imaging techniques can pinpoint brain tumors. Diagnostic tools include
computed tomography (CT or CAT scan) and magnetic resonance imaging (MRI).
Intraoperative MRI also is used during surgery to guide tissue biopsies and tumor removal.
Magnetic resonance spectroscopy (MRS) is used to examine the tumor's chemical profile and
determine the nature of the lesions seen on the MRI. Positron emission tomography (PET
scan) can help detect recurring brain tumors.
Sometimes the only way to make a definitive diagnosis of a brain tumor is through a biopsy.
The neurosurgeon performs the biopsy and the pathologist makes the final diagnosis,
determining whether the tumor appears benign or malignant, and grading it accordingly.
Brain Tumor Treatment
Brain tumors (whether primary or metastatic, benign or malignant) usually are treated with
surgery, radiation, and/or chemotherapy alone or in various combinations. While it is true
that radiation and chemotherapy are used more often for malignant, residual or recurrent
tumors, decisions as to what treatment to use are made on a case-by-case basis and depend on
a number of factors. There are risks and side effects associated with each type of therapy.
Surgery
It is generally accepted that complete or nearly complete surgical removal of a brain tumor is
beneficial for a patient. The neurosurgeon's challenge is to remove as much tumor as
possible, without injuring brain tissue important to the patient's neurological function (such as
the ability to speak, walk, etc.). Traditionally, neurosurgeons open the skull through a
craniotomy to insure they can access the tumor and remove as much of it as possible.
Another procedure that is commonly performed, sometimes before a craniotomy, is called a
stereotactic biopsy. This smaller operation allows doctors to obtain tissue in order to make an
accurate diagnosis. Usually, a frame is attached to the patient's head, a scan is obtained, and
then the patient is taken to the operating area, where a small hole is drilled in the skull to
allow access to the abnormal area. A small sample is obtained for examination under the
microscope.
In the early 1990s, computerized devices called surgical navigation systems were introduced.
These systems assisted the neurosurgeon with guidance, localization and orientation for
tumors. This information reduced the risks and improved the extent of tumor removal. In
many cases, surgical navigation systems allowed previously inoperable tumors to be excised
with acceptable risks. Some of these systems also can be used for biopsies without having to
attach a frame to the skull. One limitation of these systems is that they utilize a scan (CT or
MRI) obtained prior to surgery to guide the neurosurgeon. Thus, they cannot account for
movements of the brain that may occur intraoperatively. Investigators are developing
techniques using ultrasound and performing surgery in MRI scanners to help update the
navigation system data during surgery.
Intraoperative language mapping is considered by some as a critically important technique for
patients with tumors affecting language function, such as large, dominant-hemisphere
gliomas. This procedure involves operating on a conscious patient and mapping the anatomy

of their language function during the operation. The doctor then decides which portions of the
tumor are safe to resect. Recent studies have determined that cortical language mapping may
be used as a safe and efficient adjunct to optimize glioma resection while preserving essential
language sites.
Ventriculoperitoneal shunting may be required for some patients with brain tumors. Everyone
has cerebrospinal fluid (CSF) within the brain and spine that is slowly circulating all the time.
If this flow becomes blocked, the sacs that contain the fluid (the ventricles) can become
enlarged, creating increased pressure within the head, resulting in a condition called
hydrocephalus. If left untreated, hydrocephalus can cause brain damage and even death. The
neurosurgeon may decide to use a shunt to divert the spinal fluid away from the brain and,
therefore, reduce the pressure. The body cavity in which the CSF is diverted usually is the
peritoneal cavity (the area surrounding the abdominal organs). The shunt usually is
permanent. If it becomes blocked, the symptoms are similar to that of the original condition
of hydrocephalus and may include headaches, vomiting, visual problems, and/or confusion or
lethargy, among others.
Radiation Therapy
Radiation therapy uses high-energy X-rays to kill cancer cells and abnormal brain cells, and
to shrink tumors. Radiation therapy may be an option if the tumor cannot be treated
effectively through surgery.

Standard External Beam Radiotherapy uses a variety of radiation beams to create a

conformal coverage of the tumor while limiting the dose to surrounding normal
structures. The risk of long-term radiation injury with modern delivery methods is
very low. Newer techniques of delivery aside from 3-dimensional conformal
radiotherapy (3DCRT) include intensity-modulated radiotherapy (IMRT).

Proton Beam Treatment employs a specific type of radiation in which protons, a

form of radioactivity, are directed specifically to the tumor. The advantage is that less
tissue surrounding the tumor incurs damage.

Stereotactic Radiosurgery(such as Gamma Knife, Novalis and Cyberknife) is a

technique that focuses the radiation with many different beams on the target tissue.
This treatment tends to incur less damage to tissues adjacent to the tumor. Currently,
there is no data to suggest one delivery system is superior to another in terms of
clinical outcome, and each has its advantages and disadvantages.

Chemotherapy
Chemotherapy generally is considered to be effective for specific pediatric tumors,
lymphomas and some oligodendrogliomas. While it has been proven that chemotherapy
improves overall survival in patients with the most malignant primary brain tumors, it does so
in only in about 20 percent of all patients, and physicians cannot readily predict which
patients will benefit before treatment. As such, some physicians choose not to use
chemotherapy because of the potential side effects (lung scarring, suppression of the immune
system, nausea, etc.).

Chemotherapy works by inflicting cell damage that is better repaired by normal tissue than
tumor tissue. Resistance to chemotherapy might involve survival of tumor tissue that cannot
respond to the drug, or the inability of the drug to pass from the bloodstream into the brain. A
special barrier exists between the bloodstream and the brain tissue called the blood-brain
barrier. Some investigators have tried to improve the effect of chemotherapy by disrupting
this barrier, or by injecting the drug into the tumor or brain. The goal of another class of drugs
is not to kill the tumor cells, but, rather, to block further tumor growth. In some cases, growth
modifiers (such as breast cancer treatment drug Tamoxifen) have been used to attempt to stop
the growth of tumors resistant to other treatments.
In 1996, the U.S. Food and Drug Administration approved the use of chemotherapyimpregnated wafers, which can be applied by the neurosurgeon at the time of surgery. The
wafers slowly secrete the drug into the tumor, and the patient receives chemotherapy with the
systemic side effects of treatment.
Investigational Therapies
Many types of new therapies currently are being studied, especially on tumors for which the
prognosis is generally poor through existing conventional therapies. It is unknown whether
these therapies will work. Such therapies are given according to a protocol, and include
various forms of immunotherapy, therapy using targeted toxins, anti-angiogenesis therapy,
gene therapy and differentiation therapy. Combinations of treatments also may be able to
improve the outlook for patients, while lowering the adverse side effects.

Meningiomas
Updated June 2012
Meningiomas are the most common benign intracranial tumor. They originate from arachnoid
cap cells, which are cells within the thin, spider web-like membrane that covers the brain and
spinal cord. The arachnoid is one of three protective layers, collectively known as the
meninges, surrounding the brain and the spinal cord. The meninges also include the dura
mater and pia mater. Although the majority of meningiomas are benign, these tumors can
grow slowly until they are very large if left undiscovered, and, in some locations, can be
severely disabling and life-threatening. Most patients develop a single meningioma; however,

some patients may develop several tumors growing simultaneously in other parts of the brain
or spinal cord.
Some meningiomas are found along the dural lining in the venous sinuses of the brain and
skull base, locations where arachnoid cap cells are most abundant. The following subtypes
are based on the location of the tumor.

Cavernous Sinus Meningioma: Occurs near the area that drains deoxygenated blood
to the heart from the brain.

Cerebellopontine Angle Meningioma: Located near the margin of the cerebellum;

acoustic neuromas (vestibular schwannoma) typically are found in this area.

Cerebral Convexity Meningioma: Located on the upper surface of the brain cerebral
convexity.

Foramen Magnum Meningioma: Located near the opening at the base of the skull
through which the lower portion of the brainstem passes.

Intraorbital Meningioma: Located in or around eye sockets.

Intraventricular Meningioma: Located in the chambers through which

cerebrospinal fluid is carried throughout the brain.

Olfactory Groove Meningioma: Located along the nerves connecting the nose to the
brain.

Parasagittal/Falx Meningioma: Located adjacent to the dural fold that separates the
two brain hemispheres

Petrous Ridge Meningioma: Portion of the temporal bone (which supports the
temple) that contain sections of the organs that facilitate hearing.

Posterior Fossa Meningioma: Occurs near the back of the brain.

Sphenoid Meningioma: Located near the sphenoid bone behind the eyes.

Spinal Meningioma: Located in the spine, in some cases against the spinal cord.

Suprasellar Meningioma: Located near the area of the skull where the pituitary
gland is found.

Tentorium Meningioma: Located near where the brain connects to the brain stem, an
area known as the tentorium cerebelli.

Prevalence and Incidence

According to the Brain Science Foundation and the American Society of Clinical Oncology,
meningiomas account for about 34 percent of all primary brain tumors, and most often occur

in people between the ages of 30 and 70. Malignant meningiomas account for about two to
three percent of all meningiomas.
Types and Classification
The World Health Organization (WHO) classification of brain tumors is the most widely
utilized tool in grading tumor types. The WHO classification scheme recognizes 15 variations
of meningiomas according to their cell type as seen under a microscope. These variations are
called meningioma subtypes; the technical term for these cell variations is histological
subtypes.

World Health Organization (WHO) Meningioma Classifications

WHO Grade I
Benign

WHO Grade II
Atypical

WHO Grade III

Malignant

Meningiothelial
Fibrous (fibroblastic)
Transitional (mixed)
Psammomatous
Angiomatous
Microcystic
Secretory
Lymphoplasmacyte-rich
Metaplastic

Chordoid
Clear Cell
Atypical

Papillary
Rhabdoid
Anaplastic

Atypical meningiomas (which account for seven to eight percent of meningioma cases)
exhibit increased tissue and cell abnormalities. These tumors grow at a faster rate than benign
meningiomas and can invade the brain. Atypical meningiomas have a higher likelihood of
recurrence than benign meningiomas.
Malignant meningiomas show increased cellular abnormalities, and grow at a faster rate than
benign and atypical meningiomas. Malignant meningiomas are the most likely to invade the
brain, spread to other organs in the body and recur more often than the other two types.
Risk Factors
As noted earlier, meningiomas most often occur in people between the ages of 30 and 70.
Children are not as likely as adults to develop meningioma.
Women are more than two times as likely as men to develop a meningioma. Malignant
meningioma diagnoses, however, are three times as likely in men. Spinal meningiomas occur
10 times more frequently in women than in men.

Exposure to ionizing radiation, especially high doses, has been associated with a higher
incidence of intracranial tumors, particularly, meningiomas. There also is evidence indicating
a connection between meningiomas and low doses of radiation. The most well-known case
involves children in Israel who were given radiation for scale ringworm between 1948 and
1960. Within the U.S., dental X-rays are the most common form of exposure to ionizing
radiation. A number of studies have linked the number of full mouth dental radiographs to
increased risk of meningioma.
The genetic disorder Neurofibromatosis type 2 (NF2) is believed to put people at a higher
risk of developing meningioma. Patients with NF2 also may be more likely to develop
malignant or multiple meningiomas.
Per the Brain Science Foundation, a number of studies have suggested a correlation between
meningiomas and hormones. Such findings include the following:

Increased occurrence of meningioma in women

The detection of such hormones as estrogen, progesterone and androgen in some

meningiomas

A link between breast cancer and meningioma

A connection between meningioma growth, menstrual cycles and pregnancy

Researchers are beginning to explore the possible connection between meningioma risk and
the use of oral contraceptives and hormone-replacement therapy procedures.
Symptoms
Because meningiomas commonly are slow-growing tumors, they often do not cause
noticeable symptoms until they are quite large. Some meningiomas may remain
asymptomatic for a patient's lifetime or be detected unexpectedly when a patient has a brain
scan for unrelated symptoms. Presenting signs and symptoms depend on the size and location
of the tumor. Symptoms of meningiomas may include any of the following:

Headaches

Seizures

Change in personality or behavior

Progressive focal neurologic deficit

Confusion

Drowsiness

Hearing loss or ringing in the ears

Muscle weakness

Nausea or vomiting

Visual disorders

Symptoms can be related more specifically to the location of the meningioma. Examples
include the following:

Falx and Parasagittal: Impaired levels of brain functioning such as reasoning and
memory. If located in the middle section, it would likely cause leg
weakness/numbness or seizures.

Convexity: May cause seizures, headaches and neurological deficits.

Sphenoid: Vision problems, loss of sensation in the face or facial numbness and
seizures

Olfactory Groove: Loss of smell due to compression of the nerves that run between
the brain and the nose. If the tumor grows large enough, vision problems may occur
due to compression of the optic nerve.

Suprasellar: Vision problems due to compression of the optic nerves/chiasm.

Posterior Fossa: Facial symptoms or loss of hearing due to compression of cranial

nerves, unsteady gait and problems with coordination.

Intraventricular: May block the flow of cerebrospinal fluid, resulting in (obstructive

hydrocephalus), potentially leading to headaches, lightheadedness and changes in
mental function.

Intraorbital: Buildup of pressure in the eyes, leading to a bulging appearance and

potential loss of vision.

Spinal: Back pain or pain in the limbs caused by compression of the nerves which run
into the spinal cord.

Diagnosis
It can be difficult to diagnose meningiomas for several reasons. Because the majority of
meningiomas are slow-growing tumors and primarily affect adults, symptoms may be so
subtle that the patient and/or doctor may attribute them to the normal signs of aging. Adding
to the confusion is that some of the symptoms associated with meningiomas also can be due
to other medical conditions. Misdiagnosis is not uncommon and, in fact, may take several
years to diagnosis correctly.
When a patient presents slowly increasing signs of mental dysfunction, new seizures or
persistent headaches, or if there is evidence of pressure inside the skull (e.g. vomiting,
swelling of the optic nerve head in the back of the eye), the first step should be a thorough
neurological evaluation, followed by radiological studies, if needed.

Sophisticated imaging techniques can help diagnose meningiomas. Diagnostic tools include
computed tomography (CT or CAT scan) and magnetic resonance imaging (MRI).
Intraoperative MRI also is used during surgery to guide tissue biopsies and tumor removal.
Magnetic resonance spectroscopy (MRS) is used to examine the tumor's chemical profile and
determine the nature of the lesions seen on the MRI.
Sometimes, the only way to make a definitive diagnosis of the meningioma is through a
biopsy. The neurosurgeon performs the biopsy, and the pathologist makes the final diagnosis,
determining whether the tumor appears benign or malignant, and grading it accordingly.
Treatment Options
Surgery
Meningiomas primarily are benign tumors, frequently with defined borders and often
enabling complete surgical removal, which offers the best chance for a cure. The
neurosurgeon opens the skull through a craniotomy to enable full access to the meningioma.
The goal of surgery is to remove the meningioma completely, including the fibers that attach
it to the coverings of the brain and bone. However, complete removal can carry potential risks
that may be significant, especially when the tumor has invaded brain tissue or surrounding
veins.
Although the goal of surgery is to remove the tumor, the first priority is to preserve or
improve the patient's neurological functions. With patients for whom total removal of the
tumor carries significant risk of morbidity (any side effect that can cause decreased quality of
life), it may be better to leave some of the tumor in place and observe future growth with
regular imaging studies. In such cases, the patient will be observed over a period of time with
regular examinations and MRIs, while for other patients, radiation therapy may be deemed
the best approach. It is common for patients to undergo preoperative embolization of the
tumor to ensure safety during the surgical procedure. The embolization procedure is similar
to a cerebral angiogram except that the surgeon fills the blood vessels in the tumor with glue
to stop blood supply to the tumor.
Observation
Observation over a period of time may be the appropriate course of action in patients who
meet the following criteria:

Patients with few symptoms and little or no swelling in the adjacent brain areas.

Patients with mild or minimal symptoms who have a long history of tumors without
much negative effect on their quality of life.

Older patients with very slow-progressing symptoms.

Patients for whom treatment carries a significant risk.

Patients who choose not to have surgery after being offered alternate treatment
options.

Radiation Therapy
Radiation therapy uses high-energy X-rays to kill cancer cells and abnormal brain cells, and
to shrink tumors. Radiation therapy may be an option if the tumor cannot be treated
effectively through surgery.

Standard External Beam Radiotherapy uses a variety of radiation beams to create a

conformal coverage of the tumor while limiting the dose to surrounding normal
structures. The risk of long-term radiation injury with modern delivery methods is
very low. Newer techniques of delivery aside from 3-dimensional conformal
radiotherapy (3DCRT) include intensity-modulated radiotherapy (IMRT).

Proton Beam Treatment employs a specific type of radiation in which protons, a

form of radioactivity, are directed specifically to the tumor. The advantage is that less
tissue surrounding the tumor incurs damage.

Stereotactic Radiosurgery (such as Gamma Knife, Novalis and Cyberknife) is a

technique that focuses the radiation with many different beams on the target tissue.
This treatment tends to incur less damage to tissues adjacent to the tumor. Currently,
there is no data to suggest one delivery system is superior to another in terms of
clinical outcome. Each has its advantages and disadvantages.

Chemotherapy
Chemotherapy is rarely used to treat meningioma, except in atypical or malignant subtypes
that cannot be adequately treated with surgery and/or radiation therapy.
Outcome
In adults, the patient's age at the time of diagnosis is one of the most powerful predictors of
outcome. In general, the younger the adult, the better his or her prognosis tends to be.
There generally is a better outcome if the entire tumor is surgically removed. However, this is
not always possible due to the location of the tumor.
Data from the American Society of Clinical Oncology indicates an overall five-year survival
rate for meningioma of 69 percent. Individuals with benign meningiomas have an overall
five-year survival rate of 70 percent, while those with malignant meningiomas have an
overall five-year survival rate of 55 percent.
Additional Information
These websites offer additional helpful information on meningiomas, including treatment
options, support and more. (Note: These sites are not under the auspice of the AANS, and
their listing here should not be seen as an endorsement of these sites or their content.)

Classification of Brain Tumors

This information has been provided by the AANS/CNS Section on Tumors

A listing of most of the tumors that occur in the brain or its surrounding structures is
presented here. Sometimes a tumor may have more than one name. The descriptions of how
the tumors behave refer to the most common patterns since there are always exceptional cases
(both better and worse). Higher grade malignancies are, in general, expected to grow faster
than lower grade malignancies.
The comments on tumor treatment usually refer to conventional therapy (surgery, radiation
and cytotoxic chemotherapy) and may not include promising new, investigational or
experimental treatments. Radiosurgery may be an option for most of these tumors and its use

depends upon tumor size, location and the individual patient's case. If a tumor continues to
grow despite treatment, additional surgery may be recommended. This also depends on the
individual patient.
Clinical trials are available for many of these tumors, particularly if they are malignant or
recurrent. Specific tumors are listed alphabetically in the pages to follow. Discussions of
spinal cord tumors , familial syndromes and the remote effects of carcinoma follow the
alphabetical listing.
It should be noted that this information is provided for educational purposes only, and should
not be considered to be medical advice. Questions about a specific patient should be referred
to a qualified surgeon or other physician.
I.

Primary tumors of the brain

A.

Gliomas

Lowest grade tumors

Pilocytic astrocytoma

Subependymal giant cell astrocytoma

Protoplasmic astrocytoma

Ganglioglioma

Xanthomatous astrocytoma

Subependymoma

Lower grade malignancies

Fibrillary (gemistocytic, protoplasmic) astrocytoma

Ependymoma

Oligodendroglioma

Mixed oligo-astrocytoma

Optic nerve glioma

Higher-grade malignancies

Anaplastic astrocytoma

Anaplastic oligodendroglioma

Anaplastic mixed glioma

B.

C.

D.

E.

F.

Highest-grade malignancies

Glioblastoma multiforme

Gliosarcoma

Gliomatosis cerebri

Meningioma

Benign

Atypical

Malignant

Primitive neuroectodermal tumors (PNET)

Medulloblastoma

Ependymoblastoma

Pineoblastoma

Pituitary tumors

Pituitary adenoma

Pituitary carcinoma

Craniopharyngioma

Rathke's cleft cyst

Pineal Tumors

Pineal cyst

Pineocytoma

Pineoblastoma

Germinoma

Mixed germ cell tumor

Pineal gliomas

Pineal teratoma

Choroid plexus tumors

G.

H.

I.

J.

Choroid plexus papilloma

Choroid plexus carcinoma

Other, more benign primary tumors

Neurocytoma

Dysembroplastic neuroepithelial tumor (DNT)

Lipoma

Hemangioblastoma

Hamartoma

Teratoma

Tumors of nerves and/or nerve sheaths

Neuroma

Schwannoma

Neurofibroma

Cysts

Colloid cyst

Arachnoid cysts

Dermoid

Epidermoid

Rathke's cleft cyst

Pineal cyst

Other primary tumors, including skull base

Chondroma

Chordoma

Sarcomas

Gliosarcoma

Chondrosarcoma

K.
II.

Rhabdomyosarcoma

Primary Central Nervous System Lymphoma (PCNSL)

Metastatic brain tumors and carcinomatous meningitis

PRIMARY TUMORS OF THE BRAIN

Acoustic schwannoma
This is a schwannoma (i.e. tumor of the nerve sheath cells -- see " schwannoma ".) which
arises in the region of the eighth cranial nerve. It is located in the posterior fossa (the lower,
back part of the cranial cavity, above the neck) in the angle between the cerebellum and pons.
It is usually found in adults who develop hearing loss on one side. Tinnitus, a buzzing or
ringing sound, may also be present; other symptoms are also possible. The tumor is benign,
grows slowly, and is treated with surgery or radiosurgery. It is also known as an acoustic
neuroma. There is an Acoustic Neuroma Association, which can be reached at
www.ANAUSA.org .
Anaplastic astrocytoma
This tumor is a locally aggressive, destructive form of astrocytoma. It spreads into, or
"infiltrates"normal brain, and is considered malignant. It may occur anywhere within the
central nervous system (CNS). Conventional treatment options include surgery and radiation
therapy. Even after these treatments, it may recur. About half of the patients can be expected
to have a partial response to chemotherapy. Experimental therapies are often recommended if
the tumor continues to progress despite these standard treatments.
Anaplastic mixed glioma
This is an infiltrating glioma that grows relatively rapidly and contains more than one type of
tumor cell -- an aggressive combination of an astrocytoma and an oligodendroglioma.
Conventional treatment options include surgery and radiation therapy. The response of this
tumor to chemotherapy is intermediate between anaplastic astrocytoma and anaplastic
oligodendroglioma.
Anaplastic oligodendroglioma
An anaplastic oligodendroglioma is an aggressive (malignant) type of oligodendroglioma.
Conventional treatment options include surgery and radiation therapy. Also, most of these
tumors would be expected to respond to chemotherapy. A combination of procarbazine,
CCNU and vincristine, called "PCV" is used. Recent evidence suggests that specific
chromosomal abnormalities may predict the response to chemotherapy.
Arachnoid cyst
This is a sac of cerebrospinal fluid (CSF), which has been "trapped" outside or inside the
brain. It might also be called a "leptomeningeal cyst". Some of these cysts may date to birth;
others may arise or be diagnosed after a head injury. Usually such a cyst produces no

symptoms and is found by coincidence when a patient gets scanned for a headache or after a
head injury. However, the fluid (CSF) might be able to enter the sac more easily than exit, so
the cyst might grow over time. On rare occasions, these cysts may cause loss of neurologic
function due to stretching normal nerve cells or putting pressure on the brain, producing
seizures, or even causing a hemorrhage if a blood vessel is stretched across the cyst.
When surgery is warranted, either endoscopic (i.e. through an "endoscope" or small tube) or
open surgery can be used, depending on the surgeon's choice and the patient's situation. The
goal of the surgery is to allow for a larger passage to be created (called a fenestration)
between the cyst and the normal CSF compartments. Alternatively, the collecting fluid is
sometimes diverted by a tube (called a shunt) to another body cavity. Even after treatment,
the cyst may not appear to change in size, since the brain often adopts the shape permanently.
Similar structures that arise off the ventricular system have been called ependymal cysts.
Astrocytoma
An astrocytoma is a tumor that arises from astrocytes, which are cells that support and
nourish the neurons of the brain. Astrocytes are one type of glia; astrocytomas are a main
category of the gliomas, the tumors arising from the glia. There are many types of
astrocytomas -- please refer to the specific type, such as pilocytic astrocytoma or anaplastic
astrocytoma.
Butterfly glioma
A tumor of the glial cells (see "glioma") that has spread across the corpus callosum (which
connects the two sides of the brain) so that it involves both of the cerebral hemispheres.
Surgery is rarely helpful, other than for diagnosis (biopsy).
Brain stem glioma
A tumor arising from the glial cells and located in the brainstem, the deepest portion of the
brain. Children are often affected by this tumor. Several different pathologic types are
possible; the type determines how fast the tumor will grow. Surgery, stereotactic surgery,
radiation and/or chemotherapy may be used depending on the particular patient.
Carcinomatous meningitis
Sometimes a cancer may spread to the space around the brain or spinal cord, which contains
the meninges. Here, there are no barriers to further spread throughout this space. The function
of individual nerves and/or nerve roots (serving the head and body) becomes impaired.
Progression is usually relentless despite radiation and chemotherapy, even when the drugs are
delivered directly into the spinal fluid. These therapies are often used to attempt to stop the
disease, however. This disease is also called leptomeningeal cancer.
Chondroma

A chondroma is a tumor that arises from cartilage, usually arising at the base of the skull. It
usually grows slowly, and can become very large. The tumor can be treated by surgical
removal.
Chondrosarcoma
This is a rare, malignant tumor arising from cartilage. It may occur in the skull at the joints
between bones, or elsewhere in the skeleton. It is locally invasive, but rarely spreads to other
parts of the body. Treatment consists of surgical removal, which may be followed by
radiation therapy.
Chordoma
A chordoma is a tumor that comes from a part of the spine or skull -- called the notochord -that is left over from fetal life. It occurs at the base of the skull, or at the end of the spine.
Although this tumor is relatively grows relatively slowly and appears benign under the
microscope, the behavior is more like that of a malignant tumor because it has a tendency to
recur and spread. Treatments include surgery, radiation therapy, radiosurgery and in some
cases, chemotherapy.
Choroid plexus carcinoma
This is a malignant variety of the choroid plexus papilloma (see below). It usually occurs in
children, and can invade normal brain. A few patients have been cured with complete surgical
removal of the tumor. Otherwise, radiation and high dose chemotherapy may be used to
attempt control this tumor, because it is highly aggressive and can often be fatal.
Choroid plexus papilloma
This is a benign tumor that arises within the brain's ventricles from the cells (in the "choroid
plexus") that make spinal fluid. A choroid plexus papilloma generally becomes apparent
during infancy or early childhood. It may cause over-production (or blockage) of spinal fluid,
resulting in an accumulation of this fluid within the ventricular cavities of the brain -- this is
called "hydrocephalus". The tumor is treated with surgical removal. In rare instances, the
tumor may re-grow or spread throughout the nervous system.
Colloid cyst
This benign cyst arises in the third ventricle and is usually filled with thick, mucous-like,
fluid. Thought to be present since birth, the cyst gradually grows and eventually blocks the
normal flow the cerebral spinal fluid (CSF) from the lateral ventricles into the third ventricle.
This is one type of hydrocephalus. If it occurs gradually, the patient may develop headache,
double vision, difficult walking, memory problems or other difficulties. Sometimes a colloid
cyst may produce an abrupt fluid blockage that may cause sudden coma or even death
without prior warning. For this reason, many surgeons consider the discovery of a colloid
cyst an indication for surgery.

Management options include stereotactic aspiration, endoscopic removal (surgery through a

small tube called and "endoscope") or microsurgical removal. Removing the cyst without
causing brain damage can be difficult. Treatment should be individualized and take into
account the experience of the surgeon.
Craniopharyngioma
This is a cystic tumor that arises just above the pituitary gland. It is usually first diagnosed at
one of two ages -- childhood or the elderly. It grows relatively slowly. The principle threat is
to vision (as the tumor may compress the optic nerves) and to the function of the pituitary
gland. When very large, this tumor may threaten neurologic function or life itself due to
pressure on the brain. It may also block the drainage of spinal fluid, causing hydrocephalus.
Surgery is usually considered the first line treatment because a craniopharyngioma can
sometimes be cured at the first operation if it is completely removed. Complete removal may
not be possible however, because of "fingers" of the tumor that invades into adjacent brain
structures. In some cases, simple drainage of the cyst fluid and/or injection of radioactive
fluid can control the symptoms and growth. Radiosurgery or radiation therapy may be used in
some cases. Although this is a relatively benign tumor, the progressive growth may be
difficult to control and some patients die of this disease.
Cysts
These are not true tumors in the sense of being neoplasms (i.e. "new growths") or cancers;
nonetheless they may expand over time putting pressure on vital brain or spinal structures.
They are characterized by the accumulation of fluid or some other substance within a
confining layer. Cysts may occur in isolation, or as part of a true tumor (such as a glioma,
metastasis, or hemangioblastoma). Please see the specific type of brain cyst (or tumor) for
further information.
Dermoid or epidermoid cyst
These benign growths are due to left over (or implanted) skin tissue (dermis) within the head
or spinal canal. The skin tissue grows, producing pearly scales that would be analogous to the
shedding of dead skin. As this material has nowhere to go (unlike on the surface of the skin
where it would be shed), it accumulates and eventually forms a mass. Treatment is surgical,
although it may not be possible to cure the cyst, as often the skin cells are firmly adherent to
small nerves and blood vessels. Epidermoid cysts contain just the outer layer of skin and its
waste, while dermoids also include deeper layers of skin tissue that produce hair.
Dysembryoplastic neuroepithelial tumor (DNT)
This recently described tumor presents with intractable epilepsy in infancy and early
childhood. It is usually located in the frontal or temporal lobe of the brain. A complete
surgical resection can cure both the tumor and the seizures. Possible use of radiation is
unclear at this point.
Ependymoblastoma

See primitive neuroectodermal tumors (PNETs).

Ependymoma
This tumor arises from the ependymal cells lining the cerebrospinal fluid sacs (ventricles) of
the brain. It commonly affects children but can occur at any age. An ependymoma is a lowgrade malignancy that is best treated by removing as much as is safely possible by surgery.
Sometimes the tumor cells can spread through the spinal fluid. Radiation therapy is
sometimes used. It has not yet been determined whether or not chemotherapy is useful in
these tumors. A myxopapillary ependymoma is a benign tumor that occurs in the spine.
Epidermoid
See dermoid cyst.
Fibrillary astrocytoma
This is a tumor that grows relatively slowly, arising from astrocytes, the glial cells that
nourish and support the neurons within the brain. The tumor cells of astrocytomas mix and
coexist with normal brain tissue. While often referred to as "benign" tumors, they are more
accurately considered low-grade malignancies because they have the potential to, and usually
do, recur or turn into high-grade malignancies over time. They are often found in young
adults but can occur at any age. Other types of astrocytomas in this category are the
gemistocytic and protoplasmic varieties.
Treatment is dictated by the tumor's location and patient's symptoms. Surgery may benefit
patients whose tumors are located in parts of the brain that are not functionally important,
those with large tumors exerting pressure on the brain, or those that cause seizures. Because
tumor cells grow into the normal brain and may extend an inch or more from the visible edge
of the tumor, not every cell can be removed surgically. Cells left behind can lead to tumor
recurrence. Because of this, follow-up scans may be needed.
The role of radiation therapy is controversial for low-grade astrocytomas. Although radiation
therapy may lead to longer survival, the side effects of radiation, such as impaired thinking
and memory, limit its practical use in people with these tumors. In addition, the inability to
then use radiation treatments when the tumor progresses has led many doctors to defer
radiation treatments in patients who are not having symptoms and whose tumors are not
actively growing.
Gangliocytoma
This tumor arises from nerve cells and may also be called a ganglioneuroma. It usually is
found in children and is rarely malignant. It may cause seizures and is usually treated by
surgical removal.
Ganglioglioma

This tumor contains nerve cells and glial (supportive) cells and typically produces seizures
beginning in childhood. The seizures may be difficult to control with medicine. Surgical
removal is often feasible and is associated with good long-term tumor and seizure control for
most patients.
Germ cell tumor
There are several types of germ cell tumors, including the germinoma (see below), embryonal
carcinoma, choriocarcinoma, and yolk sac (endodermal sinus) tumors. They are relatively
uncommon, and usually occur in young adults. Usual locations are near the pineal gland or in
the suprasellar region (above the pituitary gland). These tumors may spread through the
spinal fluid. Sometimes "tumor markers" (in the blood and or spinal fluid) are used to make
the diagnosis. Treatment may include biopsy, chemotherapy and/or radiation therapy.
Germinoma
This tumor looks just like ovarian or testicular cancer under the microscope. It is the most
common of the germ cell tumors of the brain. It may spread or "seed" through the spinal
fluid. About one third of tumors in the pineal region are germinomas; however, this tumor can
occur in many locations within the brain. This tumor may cause headaches, visual problems,
hormonal disturbances and blockage of spinal fluid (hydrocephalus). It is treated with surgery
(often a biopsy for making the diagnosis), radiotherapy and sometimes chemotherapy. The
germinoma is very responsive to radiation therapy, but doctors may attempt to avoid the use
of radiation in the very young.
Glioblastoma multiforme
This is the most common primary (i.e. from the brain itself) brain tumor affecting adults.
Unfortunately, it is a rapidly growing, destructive tumor that may lead to death within
months. Under the microscope, the tumor has the features of an anaplastic astrocytoma with
the addition of areas of dead tissue (necrosis). Necrosis occurs because the tumor cells grow
faster than new blood vessels can be produced to nourish the tumor cells. Surgery, radiation
and chemotherapy are first line treatments that may prolong life to a year or so. Not all
patients are candidates for surgical removal of the tumor, for example if the tumor has already
spread into vital structures or to both sides of the brain (e.g. "butterfly glioma").
It is extremely rare for this tumor to be completely removed by surgery. This is because at the
time of first diagnosis, it has usually already spread deep into the brain. Removing the part of
the tumor that "lights up" on the MRI or CT scan does not mean that all the malignant cells
have been removed. While radiation therapy does help most patients to live a bit longer,
chemotherapy only benefits about a quarter of patients. Another treatment that has been
approved by the U.S. Food and Drug Administration (FDA) for these tumors is the surgical
implantation of wafers containing chemotherapy directly into the area of the tumor.
Radiosurgery has also been used. Immunotherapy, gene therapy and other experimental
treatments are currently under investigation for this very serious disease.
Glioma

This is a general term for any tumor that arises from tissues of the brain other than nerve cells
and blood vessels -- i.e. the "glia". There are many types of gliomas (see scheme above). The
behavior of a glioma may range from truly benign to highly malignant, depending on exactly
what type it is, and the individual patient.
Gliomatosis cerebri
The best way to think of gliomatosis cerebri is as a diffuse, infiltrating high-grade
astrocytoma without a definite mass. The tumor cells are intermixed with normal tissue
throughout the majority of the brain. Gliomatosis cerebri does not respond well to radiation
or chemotherapy, although these may be used to attempt to slow the course of the disease.
Surgery other than biopsy is also usually not an option.
Gliosarcoma
This tumor has the characteristics of a glioblastoma, but also includes malignant tissue that is
not glial in origin (sarcoma) -- which may come, for instance, from the membranes that cover
the brain. The tumor tends to be even more resistant to conventional treatments (surgery,
radiation therapy and chemotherapy) than glioblastoma. Small tumors might be partially
controlled by radiosurgery.
Hamartoma
Not a neoplasm in the usual sense, this "mass" may either be an overgrowth of tissue at its
native site, or tissue growing normally but in the wrong place. A common site is in the
hypothalamus where it may produce seizures. If treatment is needed at all, surgery or
radiosurgery are used.
Hemangioblastoma
These are usually benign tumors of blood vessels, often found in the lower part of the brain
(posterior fossa). They may be associated with cysts. Although usually solitary, multiple
hemangioblastomas can occur in Von Hippel-Lindau disease (see below), a hereditary
disorder that may also be associated with tumors of the retina, pancreas and/or kidney. This
tumor may cause dysfunction of the cerebellum (causing difficulty with walking and/or coordination, for instance). It also may block the drainage of spinal fluid, leading to
hydrocephalus. Treatment is usually by surgical removal, and can be curative. There are some
reports of successful treatment with radiosurgery. Radiation therapy or radiosurgery may also
be used if the tumor cannot be removed completely.
Hemangiopericytoma
This is a fairly rare tumor that arises from the same cells as a meningioma. It tends to recur
and spread, even to locations outside the nervous system. Treatment usually includes surgery
and radiation therapy. After these treatments, patients need to be followed with scans for local
recurrence, and also with studies to evaluate other organs, such as the liver and lungs.

Lipoma
This is a benign tumor composed of fat cells (adipose tissue). Lipomas in the head rarely
cause symptoms and are often diagnosed coincidentally. Those in the spine may cause the
spinal cord to become stretched or "tethered". Management, if necessary, is usually surgical.
Lymphoma
This is a tumor arising from lymphatic tissue (like the lymph nodes) -- which is a main
component of the body's immune system. Although lymphomas from elsewhere in the body
may spread to the brain, most cases of lymphoma affecting the brain originate within the
brain itself. Such a tumor is called a "primary CNS lymphoma". Unless a tumor is large
enough to be life threatening from its mass, treatment usually does not include surgery
(except for biopsy). Symptoms may be due to increased intracranial pressure, or due to where
the tumor (or tumors) are located more specifically within the brain. Traditionally, radiation
was the preferred treatment, however, early chemotherapy now appears to result in longer and
better survivals. One treatment for lymphoma involves disruption of the "blood-brain
barrier", so that higher doses of chemotherapy can be delivered.
In many cases, CNS lymphoma occurs in patients with AIDS (acquired immunodeficiency
syndrome) or in people infected with HIV (human immunodeficiency virus). Such patients do
not respond to treatment in the same way as patients without AIDS/HIV.
Medulloblastoma
See primitive neuroectodermal tumors (PNETs). A medulloblastoma is a PNET that arises in
or near the fourth ventricle. It is a fast-growing, invasive tumor, which may spread through
the spinal fluid. Children are usually affected, boys more commonly than girls. Treatment
includes surgical removal, staging (see PNET), and radiation and/or chemotherapy,
depending on the patient's age.
Meningioma
A meningioma is a tumor that arises from the membranes that cover the brain and surround
the central nervous system (i.e. the "linings" of the skull and spine). It is the most common
benign brain tumor in adults. Meningiomas usually grow slowly; some may not grow at all
and the doctor may choose to follow possible growth of the tumor with scans done over time.
Symptoms may include headache, seizure, loss of brain function (such as weakness,
incoordination, sensory problems), visual problems, hearing or swallowing difficulties, loss
of smell or taste, or other problems depending on the location and behavior of the mass. This
tumor may grow to be quite large before it produces symptoms. The explanation for this is
that some meningiomas grow so slowly that the brain can gradually shift or adapt to the
presence of the tumor. On the other hand, meningiomas can be found coincidentally on scans
that are being done for other reasons. Removing a small meningioma, for example, wouldn't
necessarily be expected to cure someone's headaches.

The preferred treatment for a meningioma is usually considered to be surgery if the lesion can
be largely removed at sufficiently low risk. A more complete removal is associated with a
lower risk of recurrence or progression. The surgical removal usually includes bone and/or
dura (one of the layers of the meninges) into which the tumor has spread. Replacement of
these structures may be needed. Radiation therapy, radiosurgery and/or growth-modifying
drugs may be considered as treatment options for incompletely-removed tumors or at time of
tumor recurrence or progression.
A small fraction of meningiomas may be aggressive or malignant. Malignant meningiomas
are treated with surgery, radiation therapy and possibly chemotherapy.
Metastatic brain tumors
About 1 in 4 patients with cancer will develop tumors that spread to the central nervous
system (CNS), most commonly through the blood stream to the brain. Tumors that often
spread to the brain include those originating in the lung, kidney (renal cell carcinoma), or
breast, and also melanoma. However, almost any cancer has this potential. Metastatic tumors
typically arise where the white and gray matter of the brain meet. The symptoms depend
upon the function of the affected part of the brain, but also can include headache or seizures
-- or no symptoms at all, when first detected.
The results of treatment for metastatic brain tumors was once considered to be bleak, with
survival on the order of several weeks. It has been convincingly shown, however, that
aggressive surgical management combined with radiation treatment can lead to a substantially
better outcome in some patients, both in terms of survival and quality of life. Control of a
single metastasis to the head is better when surgery is combined with radiation therapy, in
comparison to either treatment alone. The benefit of aggressive management of multiple brain
metastases is less clear, yet depending on the particular patient, surgery is sometimes
considered when there are life-threatening tumors, especially if the patient otherwise is in
good condition.
Radiosurgery has taken on an increasingly important role in the management of brain
metastases. Control is thought by many to be comparable to that achieved by surgery,
particularly when combined with conventional radiation treatments. Radiosurgery may also
prove effective in controlling some tumors that are resistant to conventional radiation (such as
melanoma and kidney cancer). The equivalence of radiosurgery to surgery for brain
metastases has not been proven, however, and there are those who believe that conventional
surgery is superior just as there are those who are advocates of radiosurgery. Treatment
should be individualized for each patient, and the patient's medical condition and extent of
cancer elsewhere in the body must be considered.
Mixed oligo-astrocytoma (mixed glioma)
This tumor shares the microscopic appearance and behavior of both astrocytomas and
oligodendrogliomas (see separate listings). Treatment options include surgery, radiation
and/or chemotherapy.

Neurinoma
Note: sometimes schwannomas or neurofibromas are referred to as neuromas or neurinomas
-- see descriptions of these other tumors.
Neuroblastoma
A neuroblastoma usually occurs outside the central nervous system. Rarely, it can occur
within the brain, more commonly in children. It usually grows rapidly, causing seizures and
other neurologic symptoms. It may also spread through the spinal fluid. Some may call it a
PNET. Surgery, radiation therapy and/or chemotherapy may be used to attempt to control it.
Neurocytoma
These tumors usually arise in the fluid sacs (ventricles) of the brain and often affect young or
middle aged adults. Because of this, it may cause hydrocephalus. Although usually slowlygrowing and benign, some neurocytomas may be malignant. This tumor is sometimes called a
"central neurocytoma". Maximal surgical resection is the treatment of choice; the tumor may
be vascular (i.e. contain many blood vessels). Radiation therapy is usually reserved for
progressive or more malignant-appearing neurocytomas.
Neurofibroma
This is a tumor of a nerve, which mixes with normal nerve tissue. It usually occurs in the
nerves outside the spine and head. Since it is mixed with normal nerve tissue, surgical
removal would be expected to lead to loss of function of that nerve. If left untreated,
however, the tumor may continue to grow, or possibly turn into malignant tumor over time.
Note: sometimes schwannomas or neurofibromas are referred to as neuromas or neurinomas.
Neuroma
These are benign growths of abnormal nerve tissue that usually occur at the site of a nerve
injury. Neuromas may be quite painful and treatment is usually medical. Surgery can be
considered for cases where pain fails to respond to medical management. Note: sometimes
schwannomas or neurofibromas are referred to as neuromas or neurinomas.
Oligodendroglioma
These tumors are thought to arise from the oligodendrocytes, which are the cells that wrap
around nerve cells and act as a form of electrical insulation for conducting the nerve
impulses. Recent evidence suggests that they may actually arise from progenitor cells that are
immature oligodendrocytes. These tumors also tend to occur in young adults and may contain
calcium deposits that appear on brain scans. They tend to be slower growing than low-grade
astrocytomas, but have the potential to turn into more aggressive tumors. Treatment is usually
surgical and radiation therapy may be recommended. Chemotherapy is considered for
progressive tumors, and usually recommended for anaplastic (i.e. more malignant)
oligodendrogliomas. (Also see anaplastic oligodendroglioma).

Optic nerve glioma

Optic nerve gliomas occur in about 10% of patients with neurofibromatosis type I (NF I -- see
below), usually during childhood. They may involve the optic nerve, optic chiasm and/or
optic tract, which are various parts of the visual system. The tumor usually does not spread to
other parts of the brain, unless it is a higher grade (more malignant) glioma. In those without
NF I, they may occur at any age. These tumors may be treated with radiation and/or
chemotherapy; surgery is also sometimes used depending on the patient.
Pilocytic astrocytoma
This tumor is usually found in a child or young adult. The name comes from the "hair-like"
appearance of the tumor cells under the microscope. The tumor is typically slowly- growing
and often can be cured by complete surgical removal. Some pilocytic astrocytomas, however,
may behave more aggressively or be surgically inaccessible. Treatment with radiation may
eventually lead to malignant progression (more rapid growth, and brain invasion).
Pineal cyst
This is generally considered to be a benign lesion, rarely causing symptoms. A pineal cyst is
often discovered coincidentally when a patient is scanned for an unrelated reason such as a
headache.
Pineal tumors
These are growths that occur in the region of the pineal gland, situated deep within the brain.
They may obstruct the cerebrospinal fluid (CSF) pathways, causing hydrocephalus, which is
treated with a surgery to "shunt" the fluid that is accumulating. Because they are difficult to
reach safely, indirect means of diagnosing these lesions may be recommended, such as
special blood and/or spinal fluid tests ("marker" studies -- see germ cell tumors) or a trial of
low dose radiation. A biopsy, or removal of the tumor might also be recommended,
depending on the patient and test results. Also see the specific type of pineal tumor, e.g.
pineocytoma.
Pineoblastoma
This is a PNET of the pineal gland (see PNET). It tends to grow rapidly and chemotherapy
and radiation therapy is usually recommended.
Pineocytoma
This is a tumor derived from pineal gland tissue, but it grows more slowly than a
pineoblastoma. It is treated with surgery and radiotherapy; radiosurgery has also been used.
Pituitary adenomas

These are common benign tumors of the pituitary gland. It is said that up to 10 percent of
people will have a pituitary adenoma (which might never have caused a problem) by the time
of their death. The pituitary gland is considered the "master gland" of the body; it produces
hormones that regulate the other glands. Some tumors secrete one or more of these hormones
in excess. Such so-called secretory pituitary adenomas are usually found due to hormonal
imbalances that affect bodily functions. They may be relatively small when detected.
Syndromes of secreting pituitary adenomas:
Name

Hormone

Symptoms

Prolactinoma

Prolactin

Women: breast milk production, change in menstrual

period.
Men: breast enlargement, impotence.

Cushing's disease

ACTH &
Cortisone

Weight gain, high blood pressure

Acromegaly

Growth
Hormone

Enlarging tissue and organs, diabetes, gigantism

Hyperthyroidism TSH &

Thyroid

Weight loss, irritability, heat intolerance

Treatment of a secretory pituitary adenoma is directed not only at controlling tumor growth,
but also at eliminating hormone over-production. True prolactinomas may often be
successfully treated with medicine alone, however many tumors may cause small elevations
of blood prolactin but not respond to this treatment. Other secreting tumors (or prolactinomas
that do not respond well to medical therapy) are treated with surgery, radiosurgery, standard
radiation therapy alone, or some combination. If unchecked, some secreting pituitary
adenomas may prove fatal.
In contrast, non-secreting pituitary adenomas are rarely detected until they grow large
enough to compress the optic nerves, resulting in some loss of vision. Again, surgery,
radiosurgery, standard radiation therapy alone or a combination of these is used for treatment.
These tumors tend to recur and may be quite difficult to control as they are often too large to
be safely cured by surgery when found. Unchecked, patients may go blind, suffer other
neurologic loss or die from pressure or infection of the brain.
Pituitary carcinoma
These are rare cancers of the pituitary gland. They are difficult to control locally and are
usually treated with surgery and some type of radiation (radiotherapy and/or radiosurgery).
They may spread to other organs of the body.
Pituitary region tumors
In addition to tumors of the pituitary gland itself, tumors or cysts may arise from nearby
structures -- see the specific type of tumor, e.g. pituitary adenoma.
Primitive neuroectodermal tumor (PNET)

This is a malignant tumor arising from cells that are believed to remain from fetal brain
development. They usually occur in early childhood but may become symptomatic in adult
life. In children, they commonly occur in or near a spinal fluid sac known as the fourth
ventricle and are called medulloblastomas. Under the microscope, a PNET is seen to consist
of densely-packed small cells that are usually blue in color (when common tissue processing
is performed). PNETs have a tendency to spread over the brain and spinal cord by way of the
spinal fluid. Treatment usually begins with surgery. Radiation therapy is used in adults, along
with chemotherapy. Young children are usually treated with chemotherapy alone, since
radiation may stunt intellectual development when given at an early age.
PNET staging is an important consideration, since the extent of treatment needed depends on
how widely the PNET has spread at the time of diagnosis. This "staging process" requires
MRI scans of the brain and spinal column, as well as examination of the spinal fluid under
the microscope to look for tumor cells.
Protoplasmic astrocytoma
This is a rare tumor which is typically slow-growing and displaces rather than mixes in with
(i.e. infiltrates) normal brain tissue. The tumor is therefore somewhat better controlled by
surgery than the more common low-grade astrocytoma. Additional treatment may include
radiation therapy or radiosurgery if the tumor progresses.
Pseudotumor cerebri (benign intracranial hypertension)
This condition does not involve a tumor at all, hence the use of the prefix "pseudo". It refers
to an increase in pressure inside the head, which can lead to loss of vision and changes in the
optic nerves -- findings which can also be caused by true brain tumors. The condition may
due to high levels of vitamin A, or to a major blood channel (sinus) shutting down inside the
head (usually due to an infection or blood clotting disorder). In other cases the cause is
unknown, but may be related to obesity. Treatment options include medical treatment of the
pressure, a shunt to drain spinal fluid and lower the pressure, and/or optic nerve sheath
decompression, to relieve pressure on the optic nerves. For obese patients, weight loss is also
needed.
Rathke's cleft cyst
This is a slowly-growing fluid-filled cyst, thought to be left over from the fetal stage. The
theory is that cells that should have migrated to the nasal/throat area became trapped in the
region of the pituitary gland. Treatment includes observation, surgery and/or radiosurgery.
Rhabdomyosarcoma
This is a malignant tumor of the muscle cells. It may arise anywhere a muscle normally
exists, such as near the eye.
Sarcoma

A sarcoma is a malignant tumor arising from connective or structural tissue such as bones,
cartilage or dura (one of the linings of the skull and spine). Sarcomas are generally malignant
but encompass a range of behaviors. They tend to be resistant to conventional treatments
(such as surgery, radiation and chemotherapy). Surgery and sometimes radiosurgery may be
useful. Please refer to the specific type of sarcoma, e.g. gliosarcoma.
Schwannoma
A schwannoma typically arises along a nerve, since it is comprised of cells that normally
provide the "electrical insulation" for the nerve cells. Usually benign, traditional management
has consisted of surgical removal. Schwannomas often displace the remainder of the normal
nerve, instead of mixing in with it (see neurofiboma). Common locations include along the
nerves in the head (especially the balance or "vestibular" nerve [also known as an acoustic
schwannoma]), in the spine, and, more rarely, along nerves that go to the limbs. Some
schwannomas in the head may be treated with radiosurgery instead of, or in addition to,
conventional microsurgery. Rarely the tumors may be malignant. Note: sometimes
schwannomas or neurofibromas are referred to as neuromas or neurinomas.
Subependymoma
Like an ependymoma, this tumor also arises from tissue that lines the ventricles. However, it
more often occurs in elderly patients. The behavior is benign. Unfortunately,
subependymomas often arise from the brainstem and surgeons may have to leave some tumor
behind if they are to avoid neurologic damage.
Subependymal giant cell astrocytoma
Generally a benign tumor, arising from tissue off the fluid sacs (ventricles) of the brain. This
tumor is often seen in children and some adults with a condition called Tuberous Sclerosis.
Tuberous Sclerosis is characterized by seizures, certain skin abnormalities of the face, and
varying degrees of mental retardation. Treatment usually consists of surgical removal, or
observation if it is not causing symptoms.
Skull base tumors
Please refer to the specific tumor, such as chordoma, meningioma, etc.
Teratoma
A benign tumor, which is a complex accumulation of normal tissue, but growing in the wrong
place. The tumor may include hair, teeth, muscle and a variety of other tissues. Treatment is
surgical.
Xanthomatous astrocytoma (pleomorphic xanthastrocytoma)
This is a rare tumor usually seen in young adults, often found in a temporal lobe after a
seizure. It tends not to infiltrate (mix) with normal brain tissue, but may spread along the

meninges. The tumor and further seizures are usually controlled by its surgical removal, but it
is intermediate in its' grade (i.e. degree of malignancy). Follow-up scans over time are
recommended. Radiation, radiosurgery and/or re-operation may be suggested for a recurrent
tumor.
ADDITIONAL INFORMATION
SPINAL TUMORS
Many of the types of tumors described above (especially glioblastoma, astrocytoma and
ependymoma) can affect the spinal cord (without being present in the brain). Metastatic
tumors often involve the bones of the spine, and/or the spinal cord. There are also tumors of
the spinal nerves (such as neurofibromas or schwannomas), lining of the spinal canal
(meningiomas), and spinal fluid compartment (meningeal carcinomatosis or gliomatosis).
Primary tumors may arise from the bones of the spine itself. Treatment is often the same as
for their cranial counterparts, although radiosurgery for the spine is in its infancy and is
generally considered investigational.
FAMILIAL SYNDROMES
In some cases, tumors affecting the nervous system are part of a constellation of problems as
opposed to being an isolated tumor. In addition, these syndromes may be passed on to the
children of the affected person. For autosomal dominant illnesses the risk of a child getting
the disorder from one affected parent is 1 in 2 or 50 per cent.
Neurofibromatosis
This disease is also known as von Recklinghausen's Disease and is generally divided into two
types:
Neurofibromatosis type I is characterized by tumors of the "peripheral" nerves that run
throughout the limbs, trunk and head. These tumors may include plexiform neurofibromas
and/or schwannomas. It is associated with particular patches of skin discoloration called
"caf-au-lait" spots. In extreme cases the skin tumors can be disfiguring. Abnormalities of
other organs and bones may also occur. Although many cases occur spontaneously, they
persons afflicted with NF I can pass it on to their offspring as an autosomally dominant trait.
Neurofibromatosis type II is often associated with schwannomas (neuromas) of both
vestibular nerves. It often leads to deafness, due either to the tumors or their treatment. Other
tumors such as gliomas of the optic nerves or hypothalamus, ependymomas or multiple
meningiomas are common. Both spontaneous and hereditary forms occur and the disease is
passed on as an autosomcal dominant disorder.
Lindau Syndrome
This disease (also known as von Hippel-Lindau disease) is characterized by the presence of
multiple hemangioblastomas, usually in the cerebellum, and the spinal cord, as well as tumors
of the retina, pancreas and kidney. Surgery can be used to treat both the brain and spinal cord

lesions while radiosurgery has been reported to control some brain lesions. Inheritance is
autosomal dominant. Information about the von Hippel-Lindau Family Alliance can be
viewed at www.vhl.org .
Tuberous Sclerosis
This syndrome often presents in childhood and is characterized by seizures, mental
retardation, specific facial lesions (hamartomas) and areas of skin discoloration. Tumors of
the lining of the ventricles (subependymal giant cell astrocytomas) may block flow of the
cerebrospinal fluid leading to a backup of the fluid called hydrocephalus. The tumors and
hydrocephalus are usually treated with surgery. Inheritance is autosomal dominant.
Remote effects of carcinoma
Some tumors produce chemicals or hormones that can directly or indirectly cause nerve cells
to die or malfunction without physically contacting those nerve cells. These chemicals are
carried through the blood stream to remote areas. Although these remote effects of carcinoma
are rare, they can be incapacitating. Treatment usually requires effective control of the tumor
producing the substances.