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Brain tumor
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Brain tumour
Classification and external
resources

CT scan of brain showing brain

cancer metastatic to the left parietal
lobe in the peri-ventricular area.

ICD-10 C71., D33.0-

D33.2

ICD-9 191, 225.0

DiseasesDB 30781

MedlinePlus 007222
000768

eMedicine emerg/334

MeSH D001932

A brain tumor is any intracranial tumor created by abnormal and uncontrolled cell
division, normally either in the brain itself (neurons, glial cells (astrocytes,
oligodendrocytes, ependymal cells), lymphatic tissue, blood vessels), in the cranial nerves
(myelin-producing Schwann cells), in the brain envelopes (meninges), skull, pituitary and
pineal gland, or spread from cancers primarily located in other organs (metastatic
tumors). Primary (true) brain tumors are commonly located in the posterior cranial fossa
in children and in the anterior two-thirds of the cerebral hemispheres in adults, although
they can affect any part of the brain. In the United States in the year 2005, it was
estimated that there were 43,800 new cases of brain tumors (Central Brain Tumor
Registry of the United States, Primary Brain Tumors in the United States, Statistical
Report, 2005 - 2006),[1] which accounted for 1.4 percent of all cancers, 2.4 percent of all
cancer deaths,[2] and 20–25 percent of pediatric cancers.[2][3] Ultimately, it is estimated that
there are 13,000 deaths per year in the United States alone as a result of brain tumors.[1]
Medical professionals are still unsure of the exact causes of brain tumors.[4] In recent
years, some scientists have drawn the conclusion that heavy use of cell phones is linked
to brain tumors.[5] There are also concerns that Wi-Fi carries the same risk.[citation needed]

Contents
[hide]

• 1 Classification
o 1.1 Primary tumors
o 1.2 Secondary tumors and non-tumor lesions
o 1.3 WHO Classification of Tumors of the Central Nervous System
• 2 Brain tumors in infants and children
• 3 Signs and symptoms
• 4 Diagnosis
• 5 Treatment and prognosis
o 5.1 Research to treatment with the vesicular stomatitis virus
• 6 =Prevention
• 7 See also
• 8 References

• 9 External links

[edit] Classification
[edit] Primary tumors

Tumors occurring in the brain include: astrocytoma, pilocytic astrocytoma,

dysembryoplastic neuroepithelial tumor, oligodendrogliomas, ependymoma, glioblastoma
multiforme, mixed gliomas, oligoastrocytomas, medulloblastoma, retinoblastoma,
neuroblastoma, germinoma and teratoma.

MRI image showing a low-grade brain glioma in a 28 year-old male. Image created on
2007-07-10.

Most primary brain tumors originate from glia (gliomas) such as astrocytes
(astrocytomas), oligodendrocytes (oligodendrogliomas), or ependymal cells
(ependymoma). There are also mixed forms, with both an astrocytic and an
oligodendroglial cell component. These are called mixed gliomas or oligoastrocytomas.
Plus, mixed glio-neuronal tumors (tumors displaying a neuronal, as well as a glial
component, e.g. gangliogliomas, disembryoplastic neuroepithelial tumors) and tumors
originating from neuronal cells (e.g. gangliocytoma, central gangliocytoma) can also be
encountered.

Other varieties of primary brain tumors include: primitive neuroectodermal tumors

(PNET, e.g. medulloblastoma, medulloepithelioma, neuroblastoma, retinoblastoma,
ependymoblastoma), tumors of the pineal parenchyma (e.g. pineocytoma,
pineoblastoma), ependymal cell tumors, choroid plexus tumors, neuroepithelial tumors of
uncertain origin (e.g. gliomatosis cerebri, astroblastoma), etc.

From a histological perspective, astrocytomas, oligondedrogliomas, oligoastrocytomas,

and teratomas may be benign or malignant. Glioblastoma multiforme represents the most
aggressive variety of malignant glioma. At the opposite end of the spectrum, there are so-
called pilocytic astrocytomas, a distinct variety of astrocytic tumors. The majority of
them are located in the posterior cranial fossa, affect mainly children and young adults,
and have a clinically favorable course and prognosis. Teratomas and other germ cell
tumors also may have a favorable prognosis, although they have the capacity to grow
very large.

Another type of primary intracranial tumor is primary cerebral lymphoma, also known as
primary CNS lymphoma, which is a type of non-Hodgkin's lymphoma that is much more
prevalent in those with severe immunosuppression, e.g. AIDS.

In contrast to other types of cancer, primary brain tumors rarely metastasize, and in this
rare event, the tumor cells spread within the skull and spinal canal through the
cerebrospinal fluid, rather than via bloodstream to other organs.

There are various classification systems currently in use for primary brain tumors, the
most common being the World Health Organization (WHO) brain tumor classification,
introduced in 1993.

[edit] Secondary tumors and non-tumor lesions

Secondary or metastatic brain tumors originate from malignant tumors (cancers) located
primarily in other organs. Their incidence is higher than that of primary brain tumors. The
most frequent types of metastatic brain tumors originate in the lung, skin (malignant
melanoma), kidney (hypernephroma), breast (breast carcinoma), and colon (colon
carcinoma). These tumor cells reach the brain via the blood-stream.

Some non-tumoral masses and lessions can mimic tumors of the central nervous system.
These include tuberculosis of the brain, cerebral abscess (commonly in toxoplasmosis),
and hamartomas (for example, in tuberous sclerosis and von Recklinghausen
neurofibromatosis).

[edit] WHO Classification of Tumors of the Central Nervous System

The website http://www.brainlife.org describes the various WHO (World Health
Organization) classifications of brain tumors, from 1979 to 2007. The most recent WHO
classification of brain tumors is on page
http://www.brainlife.org/who/2007_classification.htm.

[edit] Brain tumors in infants and children

In 2000 approximately 2.76 children per 100,000 were affected by a CNS tumor in the
United States. This rate has been increasing and by 2005 was 3.0 children per
100,000.[citation needed] This is approximately 2,500-3,000 pediatric brain tumors occurring
each year in the US. The tumor incidence is increasing by about 2.7% per year. The CNS
cancer survival rate in children is approximately 60%.[6] However, this rate varies with
the age of onset (younger has higher mortality) and cancer type.

In children under 2, about 70% of brain tumors are medulloblastoma, ependymoma, and
low-grade glioma. Less commonly, and seen usually in infants, are teratoma and atypical
teratoid rhabdoid tumor.[7] Germ cell tumors, including teratoma, make up just 3% of
pediatric primary brain tumors, but the worldwide incidence varies significantly.[8]

[edit] Signs and symptoms

Symptoms of brain tumors may depend on two factors: tumor size (volume) and tumor
location. The time point of symptom onset in the course of disease correlates in many
cases with the nature of the tumor ("benign", i.e. slow-growing/late symptom onset, or
malignant, fast growing/early symptom onset).

Many low-grade (benign) tumors can remain asymptomatic (symptom-free) for years and
they may accidentally be discovered by imaging exams for unrelated reasons (such as a
minor trauma).

New onset of epilepsy[9] is a frequent reason for seeking medical attention in brain tumor
cases.

Large tumors or tumors with extensive perifocal swelling edema inevitably lead to
elevated intracranial pressure (intracranial hypertension), which translates clinically into
headaches, vomiting (sometimes without nausea), altered state of consciousness
(somnolence, coma), dilatation of the pupil on the side of the lesion (anisocoria),
papilledema (prominent optic disc at the funduscopic examination). However, even small
tumors obstructing the passage of cerebrospinal fluid (CSF) may cause early signs of
increased intracranial pressure. Increased intracranial pressure may result in herniation
(i.e. displacement) of certain parts of the brain, such as the cerebellar tonsils or the
temporal uncus, resulting in lethal brainstem compression. In young children, elevated
intracranial pressure may cause an increase in the diameter of the skull and bulging of the
fontanelles.
Depending on the tumor location and the damage it may have caused to surrounding
brain structures, either through compression or infiltration, any type of focal neurologic
symptoms may occur, such as cognitive and behavioral impairment, personality changes,
hemiparesis, hypesthesia, aphasia, ataxia, visual field impairment, facial paralysis, double
vision, tremor etc. These symptoms are not specific for brain tumors - they may be
caused by a large variety of neurologic conditions (e.g. stroke, traumatic brain injury).
What counts, however, is the location of the lesion and the functional systems (e.g. motor,
sensory, visual, etc.) it affects.

A bilateral temporal visual field defect (bitemporal hemianopia—due to compression of

the optic chiasm), often associated with endocrine disfunction—either hypopituitarism or
hyperproduction of pituitary hormones and hyperprolactinemia is suggestive of a
pituitary tumor.

[edit] Diagnosis
Although there is no specific clinical symptom or sign for brain tumors, slowly
progressive focal neurologic signs and signs of elevated intracranial pressure, as well as
epilepsy in a patient with a negative history for epilepsy should raise red flags. However,
a sudden onset of symptoms, such as an epileptic seizure in a patient with no prior history
of epilepsy, sudden intracranial hypertension (this may be due to bleeding within the
tumour, brain swelling or obstruction of cerebrospinal fluid's passage) is also possible.

Glioblastoma multiforme and anaplastic astrocytoma have been associated in case reports
on Pubmed with the genetic acute hepatic porphyrias, including positive testing
associated with drug refractory seizures. Unexplained complications associated with drug
treatments with these tumors should alert physicians to an undiagnosed neurological
porphyria.

Symptoms include phantom odors and tastes. Often, in the case of metastatic tumors, the
smell of vulcanized rubber is prevalent.[citation needed]

Imaging plays a central role in the diagnosis of brain tumors. Early imaging methods—
invasive and sometimes dangerous—such as pneumoencephalography and cerebral
angiography, have been abandoned in recent times in favor of non-invasive, high-
resolution modalities, such as computed tomography (CT) and especially magnetic
resonance imaging (MRI). Benign brain tumors often show up as hypodense (darker than
brain tissue) mass lesions on cranial CT-scans. On MRI, they appear either hypo- (darker
than brain tissue) or isointense (same intensity as brain tissue) on T1-weighted scans, or
hyperintense (brighter than brain tissue) on T2-weighted MRI. Perifocal edema also
appears hyperintense on T2-weighted MRI. Contrast agent uptake, sometimes in
characteristic patterns, can be demonstrated on either CT or MRI-scans in most malignant
primary and metastatic brain tumors. This is because these tumors disrupt the normal
functioning of the blood-brain barrier and lead to an increase in its permeability.
Electrophysiological exams, such as electroencephalography (EEG) play a marginal role
in the diagnosis of brain tumors.

The definitive diagnosis of brain tumor can only be confirmed by histological

examination of tumor tissue samples obtained either by means of brain biopsy or open
surgery. The histological examination is essential for determining the appropriate
treatment and the correct prognosis. This examination, performed by a pathologist,
typically has three stages: interoperative examination of fresh tissue, preliminary
microscopic examination of prepared tissues, and followup examination of prepared
tissues after immunohistochemical staining or genetic analysis.

[edit] Treatment and prognosis

Many meningiomas, with the exception of some tumors located at the skull base, can be
successfully removed surgically. In more difficult cases, stereotactic radiosurgery, such as
gamma knife radiosurgery, remains a viable option.

Most pituitary adenomas can be removed surgically, often using a minimally invasive
approach through the nasal cavity and skull base (trans-nasal, trans-sphenoidal approach).
Large pituitary adenomas require a craniotomy (opening of the skull) for their removal.
Radiotherapy, including stereotactic approaches, is reserved for the inoperable cases.

Although there is no generally accepted therapeutic management for primary brain

tumors, a surgical attempt at tumor removal or at least cytoreduction (that is, removal of
as much tumor as possible, in order to reduce the number of tumor cells available for
proliferation) is considered in most cases.[10] However, due to the infiltrative nature of
these lesions, tumor recurrence, even following an apparently complete surgical removal,
is not uncommon. Several current research studies aim to improve the surgical removal of
brain tumors by labeling tumor cells with a chemical (5-aminolevulinic acid) that causes
them to fluoresce [11]. Postoperative radiotherapy and chemotherapy are integral parts of
the therapeutic standard for malignant tumors. Radiotherapy may also be administered in
cases of "low-grade" gliomas, when a significant tumor burden reduction could not be
achieved surgically.

Survival rates in primary brain tumors depend on the type of tumor, age, functional status
of the patient, the extent of surgical tumor removal, to mention just a few factors.[12]

UCLA Neuro-Oncology publishes real-time survival data for patients with this diagnosis.
They are the only institution in the United States that shows how brain tumor patients are
performing on current therapies. They also show a listing of chemotherapy agents used to
treat high grade glioma tumors.

Patients with benign gliomas may survive for many years,[13][14] while survival in most
cases of glioblastoma multiforme is limited to a few months after diagnosis if treatment is
ignored.
The main treatment option for single metastatic tumors is surgical removal, followed by
radiotherapy and/or chemotherapy. Multiple metastatic tumors are generally treated with
radiotherapy and chemotherapy. Stereotactic radiosurgery, such as Gamma Knife
radiosurgery, remains a viable option. However, the prognosis in such cases is
determined by the primary tumor, and it is generally poor.

A shunt operation is used not as a cure but to relieve the symptoms.[1] The
hydrocephalus caused by the blocking drainage of the cerebrospinal fluid can be removed
with this operation.

[edit] Research to treatment with the vesicular stomatitis virus

In 2000, researchers at the University of Ottawa, led by John Bell M.D., have discovered
that the vesicular stomatitis virus, or VSV, can infect and kill cancer cells, without
affecting healthy cells if coadministered with interferon. [15]

The initial discovery of the virus' oncolytic properties were limited to only a few types of
cancer. Several independent studies have indentified many more types susceptible to the
virus, including glioblastoma multiforme cancer cells, which account for the majority of
brain tumors.

In 2008, researchers at Yale University, led by Dr. Anthony van den Pol, artificially
engineered strains of VSV that were less cytotoxic to normal cells. This advance allows
administration of the virus without coadministration with interferon. Consequently
administration of the virus can be given intravenously or through the olfactory nerve. In
the research, a human brain tumor was implanted into mice brains. The VSV was injected
via their tails and within 3 days all tumor cells were either dead or dying.

Research on virus treatment like this has been conducted for some years, but no other
viruses have been shown to be as efficient or specific as the VSV mutant strains. Future
research will focus on the risks of this treatment, before it can be applied to humans.[16]

[edit] =Prevention
At present, there is no conclusive evidence that using cell phones or living by electrical
wires or power plants increases your risk of developing a brain tumor.[17]

[edit] See also

• List of notable brain tumor patients

[edit] References
1. ^ a b Greenlee RT, Murray T, Bolden S, Wingo PA. Cancer statistics, 2000. CA
Cancer J Clin 2000;50:7-33. PDF. PMID 10735013.
 2. ^ a b American Cancer Society. Accessed June 2000.
3. ^ Chamberlain MC, Kormanik PA. Practical guidelines for the treatment of
malignant gliomas. West J Med 1998;168:114-120. PMID 9499745.
4. ^ http://www.medicinenet.com/brain_tumor/page4.htm
5. ^ http://news.zdnet.com/2100-9584_22-195865.html
6. ^ See Table 11.2 Survival Rate
7. ^ Infantile Brain Tumors by Brian Rood for The Childhood Brain Tumor
Foundation (accessed July 2007)
8. ^ Echevarría ME, Fangusaro J, Goldman S (June 2008). "Pediatric central
nervous system germ cell tumors: a review". Oncologist 13 (6): 690–9.
doi:10.1634/theoncologist.2008-0037. PMID 18586924.
9. ^ Lopez MBS, Laws ER Jr. Neurosurgical Focus 12(2), Article 1, 2002.
10. ^ Nakamura M, Konishi N, Tsunoda S, Nakase H, Tsuzuki T, Aoki H, Sakitani H,
Inui T, Sakaki T. Analysis of prognostic and survival factors related to treatment
of low-grade astrocytomas in adults. Oncology 2000;58:108-16. PMID 10705237.
11. ^ Clinical trials in brain tumors.. Accessed June 2000.
12. ^ Nicolato A, Gerosa MA, Fina P, Iuzzolino P, Giorgiutti F, Bricolo A. Prognostic
factors in low-grade supratentorial astrocytomas: a uni-multivariate statistical
analysis in 76 surgically treated adult patients. Surg Neurol 1995;44:208-21;
discussion 221-3. PMID 8545771.
13. ^ Janny P, Cure H, Mohr M, Heldt N, Kwiatkowski F, Lemaire JJ, Plagne R,
Rozan R. (1994). Low grade supratentorial astrocytomas. Management and
prognostic factors. Cancer, 73:1937-1945. PMID 8137221.
14. ^ Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP, Lacy J,
Tsukerman L, Makuch R. (1996). Variations in the natural history and survival of
patients with supratentorial low-grade astrocytomas. Neurosurgery, 38:872-878;
discussion 878-879. PMID 8727811.
15. ^ Researchers Find Cancer-Killing Virus; July 24, 2000.
16. ^ Yale Lab Engineers Virus That Can Kill Deadly Brain Tumors; February 21,
2008.
17. ^ Brain Cancer at Mount Sinai Hospital

[edit] External links

• American Brain Tumor Association
• Brain tumor types, symptoms, diagnosis and treatment methods
• Samantha Dickson Brain Tumour Trust: research and support in the UK
• SDBTT Astro Fund: research and support for low-grade brain tumours
• Brain and Spinal Tumors: Hope Through Research (National Institute of
Neurological Disorders and Stroke)
• Brain Lesion Locator Find Lesions and Differential Diagnosis of Brain Tumors
• Frequently asked questions from the National Brain Tumor Foundation
• AFIP HandoutRadiology and Pathology of Astrocytoma
• [2]Children's Brain Tumor Foundation
• 38 LemonBrain Cancer Awareness from a Patient's Perspective
 [show]
v•d•e
Pathology: Tumor, Neoplasm, Cancer, and Oncology (C00-D48, 140-239)

[show]
v•d•e
Nervous tissue tumors (ICD-O 9350-9589) / brain tumors (C69-C72/D31-
D33, 190-192/224-225)

[show]
v•d•e
Congenital malformations and deformations of nervous system (Q00-
Q07, 740-742)

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Disclaimers The brain is a soft, spongy mass of tissue. It is protected by the bones of the
skull and three thin membranes called meninges. Watery fluid called cerebrospinal fluid
cushions the brain. This fluid flows through spaces between the meninges and through
spaces within the brain called ventricles.

A network of nerves carries messages back and forth between the brain and the rest of the
body. Some nerves go directly from the brain to the eyes, ears, and other parts of the
head. Other nerves run through the spinal cord to connect the brain with the other parts of
the body. Within the brain and spinal cord, glial cells surround nerve cells and hold them
in place.

The brain directs the things we choose to do (like walking and talking) and the things our
body does without thinking (like breathing). The brain is also in charge of our senses
(sight, hearing, touch, taste, and smell), memory, emotions, and personality.

The three major parts of the brain control different activities:

• Cerebrum - The cerebrum is the largest part of the brain. It is at the top of the brain. It uses information from
our senses to tell us what is going on around us and tells our body how to respond. It controls reading, thinking,
learning, speech, and emotions.
 The cerebrum is divided into the left and right cerebral hemispheres, which control
separate activities. The right hemisphere controls the muscles on the left side of the body.
The left hemisphere controls the muscles on the right side of the body.

• Cerebellum - The cerebellum is under the cerebrum at the back of the brain. The cerebellum controls balance
and complex actions like walking and talking.

• Brain Stem - The brain stem connects the brain with the spinal cord. It controls hunger and thirst. It also
controls breathing, body temperature, blood pressure, and other basic body functions.

The brain and nearby structures

Major parts of the brain

• 1
• 2
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 begins in cells, the building blocks that make up tissues. Tissues make up the organs of
the body.

Normally, cells grow and divide to form new cells as the body needs them. When cells
grow old, they die, and new cells take their place.

Sometimes this orderly process goes wrong. New cells form when the body does not need
them, and old cells do not die when they should. These extra cells can form a mass of
tissue called a growth or tumor.

What are benign and malignant brain tumors?

Brain tumors can be benign or malignant:

• Benign brain tumors do not contain cancer cells:

o Usually, benign tumors can be removed, and they seldom grow back.

o The border or edge of a benign brain tumor can be clearly seen. Cells from benign tumors do not
invade tissues around them or spread to other parts of the body. However, benign tumors can press
on sensitive areas of the brain and cause serious health problems.

o Unlike benign tumors in most other parts of the body, benign brain tumors are sometimes life
threatening.

o Very rarely, a benign brain tumor may become malignant.

• Malignant brain tumors contain cancer cells:

o Malignant brain tumors are generally more serious and often are life threatening.

o They are likely to grow rapidly and crowd or invade the surrounding healthy brain tissue.

o Very rarely, cancer cells may break away from a malignant brain tumor and spread to other parts of
the brain, to the spinal cord, or even to other parts of the body. The spread of cancer is called
metastasis.

o Sometimes, a malignant tumor does not extend into healthy tissue. The tumor may be contained
within a layer of tissue. Or the bones of the skull or another structure in the head may confine it. This
kind of tumor is called encapsulated.

Tumor Grade

Doctors sometimes group brain tumors by grade - from low grade (grade I)
to high grade (grade IV). The grade of a tumor refers to the way the cells
look under a microscope. Cells from high-grade tumors look more
abnormal and generally grow faster than cells What are primary brain tumors?
Tumors that begin in brain tissue are known as primary tumors of the brain. (Information
about secondary brain tumors appears in the following section.) Primary brain tumors are
named according to the type of cells or the part of the brain in which they begin.

The most common primary brain tumors are gliomas. They begin in glial cells. There are
many types of gliomas:

• Astrocytoma - The tumor arises from star-shaped glial cells called astrocytes. In adults, astrocytomas most
often arise in the cerebrum. In children, they occur in the brain stem, the cerebrum, and the cerebellum. A grade
III astrocytoma is sometimes called an anaplastic astrocytoma. A grade IV astrocytoma is usually called a
glioblastoma multiforme.

• Brain stem glioma - The tumor occurs in the lowest part of the brain. Brain stem gliomas most often are
diagnosed in young children and middle-aged adults.

• Ependymoma - The tumor arises from cells that line the ventricles or the central canal of the spinal cord. They
are most commonly found in children and young adults.

• Oligodendroglioma - This rare tumor arises from cells that make the fatty substance that covers and protects
nerves. These tumors usually occur in the cerebrum. They grow slowly and usually do not spread into
surrounding brain tissue. They are most common in middle-aged adults.

Some types of brain tumors do not begin in glial cells. The most common of these are:

• Medulloblastoma - This tumor usually arises in the cerebellum. It is the most common brain tumor in children.
It is sometimes called a primitive neuroectodermal tumor.

• Meningioma - This tumor arises in the meninges. It usually grows slowly.

• Schwannoma - A tumor that arises from a Schwann cell. These cells line the nerve that controls balance and
hearing. This nerve is in the inner ear. The tumor is also called an acoustic neuroma. It occurs most often in
adults.

• Craniopharyngioma - The tumor grows at the base of the brain, near the pituitary gland. This type of tumor
most often occurs in children.

• Germ cell tumor of the brain - The tumor arises from a germ cell. Most germ cell tumors that arise in the brain
occur in people younger than 30. The most common type of germ cell tumor of the brain is a germinoma.

• Pineal region tumor - This rare brain tumor arises in or near the pineal gland. The pineal gland is located
between the

from low-grade tumors