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Brain tumors - primary

  Gliomas; Medulloblastomas

An in-depth report on the causes, diagnosis, and treatment of primary brain tumors.


Primary Brain Tumors


  • Gliomas
  • Meningiomas
  • Medulloblastomas
  • Pituitary adenomas
  • Central nervous system lymphomas



Risk Factors




  • Type of tumor
  • Tumor grade
  • Person's age
  • Person's ability to function
  • How far the tumor has spread










They can be benign (noncancerous, meaning that they DO NOT spread elsewhere or invade surrounding tissue) or malignant (cancerous). Cancerous (malignant) brain tumors are classified as either primary or secondary tumors. Primary tumors start in the brain. Secondary tumors spread to the brain from another site such as the breast or lung by a process called metastasis, and they are also called metastatic brain tumors. This report focuses on primary malignant brain tumors.

Primary Brain Tumors


Benign brain tumors

Benign tumors represent half of all primary brain tumors. Their cells look relatively normal, grow slowly, and DO NOT spread (metastasize) to other sites in the body or invade brain tissue. Benign tumors can still be serious if they are in vital areas in the brain. They can be life threatening if by their growth they exert pressure on sensitive nerve tissue or increase pressure within the skull. Fortunately, most benign brain tumors can be successfully treated.

Malignant brain tumors

A primary malignant brain tumor is cancer that originates in the brain itself. In most cases, their cell origin is not neurons (nerve cells), but other kinds of cells found in the brain. Although primary brain tumors often transmit cancerous cells to other sites in the central nervous system (the brain or spine), they rarely spread to other parts of the body.

Brain tumors are generally named and classified according to either:

  • The type of brain cells from which they originate
  • The location in which the cancer develops

The biologic diversity of these tumors can make classification difficult.

Secondary (Metastatic) Malignant Brain Tumors

A secondary brain tumor is metastatic cancer. Secondary brain tumors occur when cancer cells spread (metastasize) to the brain from a primary cancer in another part of the body. Most often, cancers that spread to the brain originate in the lung, breast, kidney, or skin. Secondary tumors are about 3 times more common than primary tumors of the brain.

All secondary (metastatic) brain tumors are malignant (cancerous). This report discusses primary malignant brain tumors.

Primary Glioma Brain Tumors




Gliomas may develop from several types of glial cells.



  • Grade II. Diffuse astrocytoma (also called low-grade astrocytoma) typically occurs in men and women ages 20 to 60.
  • Grade III. Anaplastic astrocytoma typically occurs in adults ages 30 to 60 and is more common among men than women.
  • Grade IV. Glioblastoma multiforme (GBM), also called glioblastoma, accounts for about 50% of all astrocytomas and is one of the deadliest types of brain tumors. These highly aggressive malignant tumors grow rapidly. They are most common in older adults (ages 50 to 70), particularly men. Only about 10% of childhood brain tumors are glioblastomas.




Mixed gliomas



Location of the tumor


  • Brain stem gliomas develop in the lowest portion of the brain. The brain stem connects the cerebrum (the largest part of the brain) to the spinal cord. Between 10% to 20% of brain tumors in children are brain stem gliomas. Most of these tumors are astrocytomas.



Primary Non-Glioma Brain Tumors

Malignant types of non-glioma brain tumors include:

  • Medulloblastomas. Are always located in the cerebellum, which is at the base and toward the back of the brain. These fast-growing high-grade tumors represent about 15% to 20% of pediatric brain tumors and 20% of adult brain tumors.
  • Pituitary tumors (also called pituitary adenomas). Comprise about 10% of primary brain tumors and are often benign, slow-growing masses in the pituitary gland. They are more common in women than men.

  • Central nervous system (CNS) lymphomas. Can affect both people with healthy immune systems and those who are immunocompromised due to other medical conditions (recipients of organ transplants, people infected with HIV). CNS lymphomas occur most often in the cerebral hemisphere but can also develop in cerebrospinal fluid, eyes, and spinal cord.

Benign types of non-glioma brain tumors include:

  • Meningiomas. Are usually benign tumors that develop in the membranes that cover the brain and spinal cord (the meninges).




Only 5% to 10% of primary brain tumors are associated with genetic disorders. These inherited conditions and associated genes include:

  • Turcot syndrome (APC gene)
  • Tuberous sclerosis (TSC1 and TSC2 genes)
  • Li-Fraumeni syndrome (TP53 gene)



Risk Factors





Most brain tumors in adults occur between the ages of 65 to 79. Brain tumors also tend to occur in children younger than age 8. In children, malignant brain tumors are the leading cause of death from solid tumors (non-blood cell cancers).


The risk for primary brain tumors, especially gliomas, is higher in whites than in people of other races.

Environmental or Occupational Risk Factors

Exposure to ionizing radiation, usually from radiation therapy, is the only environmental risk factor that has been definitively linked to brain tumors. People who receive radiation therapy to the head during cancer treatment have an increased risk of developing brain tumors 10 to 15 years later. Workers in the nuclear industry are also at increased risk.

Despite much research, there is no evidence that electromagnetic field exposure from power lines or household appliances poses any risk. Several epidemiological studies have examined the cancer risk of wireless devices such as cell phones, which emit radiofrequency (RF) energy. To date, studies DO NOT show a clear association between cell phone use and common brain tumors like gliomas and meningiomas. However, more research is needed to examine potential long-term effects, particularly for children and adolescents.


Medical Conditions



Advances in surgical and radiation treatments have significantly extended survival for people with brain tumors. These new treatments can often help reduce the size and progression of malignant gliomas.

Survival Rates


  • Tumor grade
  • Person's age
  • Person's ability to function
  • How far the tumor has spread

Some types of tumors have relatively good survival rates. Five-year survival rates for people with ependymoma and oligodendroglioma are, respectively, up to 91% and 85% for people ages 20 to 44, and 84% and 64% for people ages 55 to 64. For children, the survival rate for these brain tumors are about 75% and 95%.

Glioblastoma multiforme has the worst prognosis with 5-year survival rates of only about 17% for people ages 20 to 44 and 4% for people age 55 to 64. The 5-year survival rate for children is about 20%. Survival rates tend to be highest for younger people and decrease with age.

Grading Tumors


Grading is based on the appearance of the tumor cells as seen under a microscope:

  • Higher-grade (III and IV) tumor cells are abnormal appearing and are more diffuse, which indicates more aggressive behavior and a poor outlook. High-grade brain tumors usually require surgery, radiotherapy, chemotherapy, and possibly investigational treatments.






Headaches caused by brain tumors vary depending on the tumor's location and other factors. Such headaches may have similar symptoms to other less serious types of headaches such as migraine, tension headache, or others.

Symptoms that may be associated with brain tumors include a headache that is:

  • Steady and worse upon waking in the morning, then goes away within a few hours
  • Persistent during sleep and accompanied by at least one other symptom (such as vomiting or confusion)
  • Accompanied by double vision, weakness, or numbness
  • Worse with coughing or exercise or with a change in body position

Gastrointestinal Symptoms

Gastrointestinal symptoms, including nausea and vomiting, are common.


About half of people with brain tumors have a seizure. It is a common symptom of brain tumors in older adults. A seizure may be the first symptom of a brain tumor in up to half of affected people.

Brain tumors may cause partial or generalized seizures:

  • Partial seizures are caused by localized tumors that affect one area of the brain. In this case, a person does not lose consciousness but may have confusion, jerking movements, tingling, or odd mental and emotional events.
  • Generalized seizures, cause loss of consciousness and are often associated with jerking movements of the whole body. In people with brain tumors, most generalized seizures start off in a specific area of the brain and then spread to involve the whole brain (partial seizures with secondary generalization).

Mental Changes


  • Memory loss
  • Impaired concentration
  • Problems with reasoning
  • Changes in personality and behavior
  • Increased sleep

Other Significant Symptoms

Other symptoms may include:

  • Gradual loss of movement or sensation in an arm or leg
  • Unsteadiness and problems with balance
  • Blurred or double vision
  • Hearing loss with or without dizziness
  • Difficulty speaking and understanding words


A neurological exam is usually the first test given when a person complains of symptoms that suggest a brain tumor. The exam includes checking eye movements, hearing, sensation, muscle strength, sense of smell, and balance and coordination. The doctor will also test mental state and memory.

Imaging Techniques


Magnetic resonance imaging (MRI)

MRI is the standard imaging procedure for diagnosing a brain tumor. It provides pictures from various angles that can help doctors to construct a 3-dimensional image of the tumor. It gives a clear picture of tumors near bones, smaller tumors, brain stem tumors, and low-grade tumors. MRI is also useful during surgery to show tumor bulk, for accurately mapping the brain, and for detecting response to therapy.

An MRI of the brain creates a detailed image of the complex structures in the brain. An MRI creates a 3-dimensional picture of the brain, which allows doctors to more precisely locate problems such as tumors or aneurysms.

Computed tomography (CT)

Uses a sophisticated x-ray machine and a computer to create a detailed picture of the body's tissues and structures. It is not as sensitive as an MRI in detecting small tumors, brain stem tumors, and low-grade tumors. However, it is useful in certain situations. Injection with a contrast material can make it easier to see abnormal tissues.

A CT scan helps locate the tumor and can sometimes help determine its type. It can also help detect swelling, bleeding, and associated conditions. CT is also used to evaluate the effectiveness of treatments and monitor for tumor recurrence.

Positron emission tomography (PET)



Other advanced or investigational imaging techniques include:

  • Magnetic resonance angiography (MRA) evaluates blood flow. MRA may be used to plan surgical removal of a tumor suspected of having a large blood supply.

Lumbar Puncture (Spinal Tap)




In some cases, such as with brain stem gliomas, a standard biopsy might be too hazardous because removing any healthy tissue from this area can affect vital functions. In these cases, surgeons can use alternative techniques such as needle biopsy and stereotactic biopsy. Stereotactic biopsy is a computer-directed type of needle biopsy that uses images provided from MRI or CT scans to provide precise information on the tumor's location.

Wada Test

The Wada test is sometimes done before surgery. Medicine is given through a catheter and into each side of the brain, one side at a time. The person is asked to talk and perform other functions while each side of the brain is under the effect of the medicine. This enables the doctors to know which side of the brain controls certain brain functions, particularly language, which could be affected by surgery to remove the tumor.


Treatment of brain tumors can involve surgery, radiation therapy, and chemotherapy. Brain tumors are best treated by a team that includes:

  • Neuro-oncologist
  • Neurosurgeon
  • Medical oncologist
  • Radiation oncologist
  • Neurologist
  • Other health care providers, such physical therapists, and social workers

Standard Treatments


The intensity, combination, and sequence of treatments depend on:

  • The brain tumor type (there are over 100 types of brain tumors)
  • The brain tumor size and location
  • The person's age, health status, and medical history.


With some very slow-growing cancers, such as those that occur in the midbrain or optic nerve pathway, the person may be closely observed and not treated until the tumor shows signs of growth.

Other Treatments

TTF therapy

Tumor Treating Fields (TTF) therapy uses a low-intensity electrical field to disrupt the rapid division of cancer cells. In 2011, the FDA approved the NovoTTF-100A (now called Optune) system to treat adults with newly diagnosed glioblastoma multiforme (GBM) that has been removed as much as possible, or GBM that has recurred or progressed despite chemotherapy and radiation. This device uses electrodes placed on the person's scalp to deliver alternating electrical fields to the tumor site. The device is portable and can be used at home.

Investigational treatments

People with brain tumors can enroll in clinical trials that are researching new treatments.


Surgery is usually the first step in treating most brain tumors. In some cases, such as most brain stem gliomas and other tumors located deep inside the brain, it may be too dangerous to perform surgery.



The standard surgical procedure is called craniotomy.

  • The tumor is located and then removed.
Craniotomy - series




Shunt Placement

Sometimes a brain tumor can create blockage and cerebrospinal fluid accumulates excessively in the skull, causing increased intracranial pressure. In these cases, a surgeon may implant a ventriculoperitoneal (VP) shunt to help drain the fluid.


Risks and Complications of Surgery

The most serious concern of brain surgery is preserving brain function. Neurosurgeons will try to be conservative in their approach so as to limit removing tissue that may cause a loss of function. Bleeding and blood clots are other complications.





Radiotherapy after surgery


Radiotherapy when surgery is not appropriate


Radiotherapy and chemotherapy (radiochemotherapy)

Combining chemotherapy with radiotherapy is beneficial for some people with high-grade tumors.

Conventional Radiotherapy


Conventional external-beam radiation techniques include:

  • Three-dimensional conformal radiation therapy (3D-CRT) uses computer-generated imaging scans to map the tumor's location. Radiation beams are then used that conform to the 3-dimensional shape of the tumor.

Stereotactic Radiosurgery


Benefits of stereotactic radiosurgery include:

  • The radiation is usually delivered in a single session, but it can be repeated if necessary. Stereotactic radiosurgery can also be used as a "booster" after conventional radiation therapy.
  • Combining stereotaxy with techniques that evaluate speech and other mental functions in people who are awake during the procedure can allow removal of brain tissue with a lower risk for complications in areas that affect such functioning.


  • First, a local anesthetic is given. In the standard operation, the person's head is immobilized by screwing a device known as a stereotactic frame into the skull. "Frameless" stereotaxy uses a mesh face mask. The frame or mask is removed when the procedure is completed.
  • A 3-dimensional map, usually using magnetic resonance imaging (MRI) scans, is made of the brain.

Once the preliminary planning stage has been completed, treatment begins. Advanced machines, such as gamma knife and adapted linear accelerator (LINAC), are used with stereotaxy to deliver very focused beams of radiation. Actual treatment takes about 15 minutes to 2 hours:

  • Gamma knife uses gamma rays that are sent from multiple points to converge at a single point on the tumor. Although each gamma-ray beam is a very low dosage, when the beams converge, the intensity and destructive power is very high. The gamma knife is used only on very small tumors. It is generally used as a booster after standard radiation, surgery, chemotherapy, or combinations.
  • LINAC produces protons (positively-charged atomic particles) in patterns that are matched to the tumor shape. The person is positioned on a bed that can be moved to allow flexible positioning. It allows treatment over multiple sessions of small doses (fractionated stereotactic radiotherapy), instead of a single session. This means that larger tumors can be treated.

Drugs Used With Radiation



Side Effects of Radiation

Common side effects


  • Hair loss
  • Fatigue
  • Headaches
  • Nausea and vomiting


Tissue injury

Radiation necrosis (total destruction of nearby healthy tissue) occurs in some people treated with intensive radiation. Radiation necrosis can cause brain swelling and reduction in mental functions. The condition is treated with steroids. If steroids prove ineffective, surgery may be required to remove the damaged tissue.

New tumors





Chemotherapy uses drugs to kill or alter cancer cells. Chemotherapy is not an effective initial treatment for low-grade brain tumors. This is mostly because many drugs have a hard time passing into the brain due to the blood-brain barrier that protects the brain from harmful chemicals. In addition, not all types of brain tumors respond to chemotherapy. In general, chemotherapy for brain tumors is usually administered following surgery or radiation therapy.

The type of drug determines how it is administered:

  • Local delivery. Drugs are placed within or around the brain tumor.

Newer delivery methods to overcome some of these problems include:

  • Intrathecal chemotherapy. Delivers chemotherapeutic drugs directly into the spinal fluid.
  • Intra-arterial chemotherapy. Delivers high-dose chemotherapy into arteries in the brain using tiny catheters.
  • Convection-enhanced delivery (CED). CED involves placing catheters into the brain tumor or nearby brain tissue to deliver slowly and continuously a cancer drug over several days.

Chemotherapy Drugs and Regimens


Temozolomide (Temodar, generic)

Temozolomide is used for adults with anaplastic astrocytoma that did not respond to other treatments. It is used during and after radiation therapy for people newly diagnosed with glioblastoma multiforme.

The current first-line treatment for people with glioblastoma is combined radiotherapy and temozolomide, followed by monthly doses of temozolomide after radiation treatment ends. The drug may work best for people with a specific genotype.


Lomustine (CeeNU, Gleostine)

Lomustine is used alone or in combination with other chemotherapy for several types of recurrent brain cancer. It is usually administered after surgery or radiotherapy.

Lomustine can cause suppression of bone marrow function and contribute to bleeding or severe infections. Blood counts should be monitored weekly for at least 6 weeks after each dose. Other potential toxic effects are on the lungs, kidney, or liver.

Carmustine (BCNU, BiCNU)


If carmustine is administered intravenously, side effects may include:

  • Nausea and vomiting
  • Fatigue
  • Respiratory problems
  • Lung scarring (pulmonary fibrosis)



PCV drug regimen

PCV is an abbreviation for a chemotherapy regimen that combines procarbazine (Matulane), lomustine (CeeNU, CCNU), and vincristine (Oncovin, generic). PCV is commonly used to treat oligodendrogliomas and mixed oligoastrocytomas. The regimen has shown good results following radiation in treating people with low-grade gliomas. The drugs may be used alone or in other combinations.


  • Beer
  • Red wine
  • Aged cheeses
  • Processed or pickled meats
  • Soybean products
Platinum-based drugs


Other chemotherapy drugs


Biologic Drugs (Targeted Therapy)

Traditional chemotherapy drugs can be effective, but they can cause severe side effects because they DO NOT distinguish between healthy and cancerous cells. Targeted biologic therapies work on a molecular level by blocking specific mechanisms associated with cancer cell growth and division.

Because they selectively target cancerous cells, biologic drugs may cause less severe side effects. In addition, these drugs hold the promise of creating options for more individualized cancer treatment based on a person's genotype.

Bevacizumab (Avastin)

Bevacizumab (Avastin) is a biologic drug that blocks the growth of blood vessels that feed tumors (a process called angiogenesis). It is approved for the treatment of glioblastoma in people whose brain cancer has continued to progress after prior treatment with chemotherapy and radiation. Bevacizumab was the first targeted therapy approved for brain tumors.

Investigational targeted therapies

Many different targeted therapies are being tested in clinical trials. They include:

  • Monoclonal antibodies targeting other molecules. Examples include cetuximab or nimotuzumab.

Treatment of Complications

Peritumoral Edema

Edema is swelling due to excess fluid retention. Normal brain tissue next to a tumor can swell and cause more pressure in addition to that from the tumor.

Edema associated with some tumors, particularly medulloblastomas, interferes with the flow of cerebrospinal fluid and causes hydrocephalus (accumulation of fluid in the skull), which in turn causes a build-up fluid in the ventricles (the cavities) in the brain.

Symptoms of peritumoral edema include:

  • Nausea and vomiting
  • Severe headaches
  • Lethargy
  • Difficulty staying awake
  • Seizures
  • Visual impairment
  • Irritability
  • Fatigue


Corticosteroids (commonly called steroids), such as dexamethasone (Decadron), are used to treat peritumoral edema. Side effects include high blood pressure, elevated blood sugar (glucose) levels, mood swings, increased risk of infection, stronger appetite, facial swelling, and fluid retention, among others.



Seizures are common in brain tumors, with younger people having higher risks than older ones. Anti-seizure (anticonvulsant) medications, such as lamotrigine or valproic acid, may treat seizures and are helpful in preventing recurrence.

These drugs are not useful in preventing a first seizure, however, and they should not be used routinely to treat people with newly diagnosed brain tumors. Anti-seizure medications should be used only for people who have experienced a seizure.

Anti-seizure medications can interact with some of the chemotherapies used to treat brain cancers, including paclitaxel, irinotecan, interferon, and retinoic acid. People should discuss these interactions with their doctors.

Muscle Weakness

Brain tumors often impair muscle function, including speech and fine motor skills.

Physical and occupational therapists can assist with rehabilitation for muscle weakness. They can also advise on home modifications, assistive devices, and adaptive tools. Speech therapists can help address problems with talking and word comprehension.

Cognitive Function

Brain tumors can affect many aspects of cognitive function. They can cause difficulties with:

  • Memory
  • Processing of information
  • Attention and concentration
  • Visual perception skills

People may face challenges with planning, insight, initiative, and organizational competencies. Cognitive rehabilitation therapists can assist in recovery with various techniques.

Children younger than age 7 (and particularly those younger than age 3 years) have some of the greatest risks for developing long-term cognitive deficits. These problems may result from the tumor and from treatments such as cranial radiation therapy and chemotherapy that penetrates the blood-brain barrier. Parents should make sure that children receive appropriate supportive services and educational accommodation at their schools.

Depression and Stress

Brain tumors can cause mood, personality, and emotional changes. You should discuss emotional as well as physical issues with your health care team. Depression can be treated medically and through psychotherapy. Relaxation techniques can be helpful. Support groups can also have great benefit for both patients and their families.


  • National Cancer Institute -- www.cancer.gov
  • American Cancer Society -- www.cancer.org
  • ASCO Cancer.Net -- www.cancer.net
  • American Brain Tumor Association -- www.abta.org
  • Children's Brain Tumor Foundation -- cbtf.org
  • Musella Foundation for Brain Tumor Research and Information -- virtualtrials.com
  • National Brain Tumor Society -- braintumor.org
  • RadiologyInfo.org -- www.radiologyinfo.org



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Review Date: 4/15/2019  


