A primary brain tumor is a group (mass) of abnormal cells that start in the brain. This article focuses on primary brain tumors in children.

Glioblastoma multiforme - children; Ependymoma - children; Glioma - children; Astrocytoma - children; Medulloblastoma - children; Neuroglioma - children; Oligodendroglioma - children; Meningioma - children; Cancer - brain tumor (children)

The cause of primary brain tumors is unknown. Primary brain tumors may be:

• Benign (not cancerous)
• Invasive (spread to nearby areas)
• Located in only a small area
• Malignant (cancerous)

Brain tumors are classified depending on the exact site of the tumor, the type of tissue involved, and whether or not it is cancerous. Brain tumors can directly destroy brain cells. They may also indirectly damage cells by pushing on other parts of the brain. This leads to swelling and increased pressure within the skull.

Tumors may occur at any age, but many specific tumors are more common at a certain age. However, most brain tumors are rare in the first year of life. Some of the most common childhood brain tumors include:

• Astrocytomas
• Brain stem gliomas
• Ependymomas
• Medulloblastomas

SPECIFIC TUMOR TYPES

Astrocytomas are usually noncancerous, slow-growing cysts. They most commonly develop in children ages 5 - 8.

Brainstem gliomas occur almost only in children. The average age of development is about 6 years old. The tumor may grow very large before triggering symptoms.

Ependymomas make up about 8 - 10% of pediatric brain tumors. The tumors are located in tiny passageways (ventricles) in the brain, and block the flow of cerebrospinal fluid (CSF).

Medulloblastomas are the most common type of childhood brain cancer. They occur more often in boys than girls, usually around age 5. Most medulloblastomas occur before the age of 10.

Symptoms may be subtle and only gradually become worse, or they may occur very quickly.

Headache is probably the most common symptom. Headache symptoms that may occur with brain tumors include:

• Double vision, weakness, or numbness
• Headache that gets worse when waking up in the morning, and then clears up within a few hours
• Headaches that may get worse with coughing or exercise, or with a change in body position
• Headaches that occur while sleeping and with at least one other symptom (such as vomiting or confusion)

Patients with brain tumors may have a seizure.

Sometimes the only symptoms of brain tumors are mental changes, which may include:

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

Other possible symptoms are:

• Gradual loss of movement or feeling in an arm or leg
• Hearing loss, with or without dizziness
• Speech difficulty
• Unexpected vision problem (especially if it occurs with a headache), including vision loss (usually of peripheral vision) in one or both eyes, or double vision
• Unsteadiness and problems with balance

The health care provider will perform a physical exam. Infants may have the following physical signs:

• Bulging fontanelles
• Enlarged eyes
• No red reflex in the eye
• Positive Babinski's reflex
• Separated sutures

The following tests may be used to detect a brain tumor and identify its location:

• CT-guided biopsy (may confirm the exact type of tumor)
• CT scan of the head
• EEG
• Examination of the cerebral spinal fluid (CSF)
• MRI of the head

Early treatment often improves the chance of a good outcome. Treatment, however, depends on the size and type of tumor and the general health of the child.

The following are treatments for specific types of tumors:

Astrocytoma -- The main treatment is surgery to remove the tumor.

Brainstem gliomas -- Surgery is usually not possible because of the tumor's location in the brain. Radiation and chemotherapy are used to shrink the tumor and prolong life.

Ependymomas -- Treatment may include surgery, radiation therapy, and chemotherapy.

Medulloblastomas -- Surgery alone does not cure this type of cancer. Chemotherapy and radiation are often used in combination with surgery.

The goals of treatment may be to cure the tumor, relieve symptoms, and improve brain function or the child's comfort.

Surgery is necessary for most primary brain tumors. Some tumors may be completely removed. Those that are deep inside the brain or that enter brain tissue may be debulked instead of entirely removed. Debulking is a procedure to reduce the tumor's size.

In cases where the tumor cannot be removed, surgery may help reduce pressure and relieve symptoms.

Radiation therapy and chemotherapy may be used for certain tumors.

Other medications used to treat primary brain tumors in children include:

• Corticosteroids such as dexamethasone to reduce brain swelling
• Diuretics such as urea or mannitol to reduce brain swelling and pressure
• Anticonvulsants such as phenytoin or levetiracetam to reduce seizures
• Pain medications

Comfort measures, safety measures, physical therapy, occupational therapy, and other such steps may be required to improve quality of life. Counseling, support groups, and similar measures may be needed to help in coping with the disorder.

For additional information, see cancer resources.

About 3 out of 4 children survive at least 5 years after being diagnosed with a brain tumor.

Long-term brain and nervous system problems may result from the tumor itself, or from treatment.

• Children may have problems with attention and concentration, memory, processing information, planning, insight, initiative or desire to do things, and the ability to stay organized and on-task.
• Children younger than age 7 (and especially those younger than age 3) appear to have the greatest risk for these problems.

Parents need to make sure that children receive needed support services at home and school.

• Brain herniation (often fatal)
• Loss of ability to interact or function
• Permanent, worsening, severe brain and nervous system (neurological) problems
• Side effects related to chemotherapy and radiation
• Tumor returns (relapse)

Call a health care provider if a child develops persistent headaches or other symptoms of a brain tumor.

Go to the emergency room if a child has a seizure that is unusual or suddenly develops stupor (reduced alertness), vision changes, or speech changes.

Buckner JC, Brown PD, O'Neill BP, Meyer FB, Wetmore CJ, Uhm JH. Central nervous system tumors. Mayo Clin Proc. 2007;82(10):1271-1286.

National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Central nervous system cancers. V.2.2009.

Glioblastoma multiforme - adults; Ependymoma - adults; Glioma - adults; Astrocytoma - adults; Medulloblastoma - adults; Neuroglioma - adults; Oligodendroglioma - adults; Meningioma - adults; Cancer - brain tumor (adults)

A primary brain tumor is a group (mass) of abnormal cells that start in the brain. This article focuses on primary brain tumors in adults.

See also:

• Brain tumor - metastatic (cancer that has spread to the brain from elsewhere in the body)
• Brain tumor - children

Primary brain tumors include any tumor that starts in the brain. Primary brain tumors can arise from the brain cells, the membranes around the brain (meninges), nerves, or glands.

Tumors can directly destroy brain cells. They can also damage cells by producing inflammation, placing pressure on other parts of the brain, and increasing pressure within the skull.

The cause of primary brain tumors is unknown. There are many possible risk factors that could play a role.

• Radiation therapy to the brain, used to treat brain cancers, increases the risk for brain tumors up to 20 or 30 years afterwards.
• Exposure to radiation at work or to power lines, as well as head injuries, smoking, and hormone replacement therapy have NOT yet been shown to be factors.
• The risk of using cell phones is hotly debated. However, most recent studies have found that cell phones, cordless phones, and wireless devices are safe and do not increase the risk.
• Some inherited conditions increase the risk of brain tumors, including neurofibromatosis, Von Hippel-Lindau syndrome, Li-Fraumeni syndrome, and Turcot syndrome.

SPECIFIC TUMOR TYPES

Brain tumors are classified depending on the exact site of the tumor, the type of tissue involved, whether they are noncancerous (benign) or cancerous (malignant), and other factors. Sometimes, tumors that start out being less invasive can become more invasive.

Tumors may occur at any age, but many types of tumors are most common in a certain age group. In adults, gliomas and meningiomas are most common.

Gliomas come from glial cells such as astrocytes, oligodendrocytes, and ependymal cells. The gliomas are divided into three types:

• Astrocytic tumors include astrocytomas (less malignant), anaplastic astrocytomas, and glioblastomas (most malignant).
• Oligodendroglial tumors also can vary from less malignant to very malignant. Some primary brain tumors are made up of both astrocytic and oligodendrocytic tumors. These are called mixed gliomas.
• Glioblastomas are the most aggressive type of primary brain tumor.

Meningiomas are another type of brain tumor. These tumors:

• Occur most commonly between the ages of 40 - 70
• Are much more common in women
• Are usually (90% of the time) benign, but still may cause devastating complications and death due to their size or location. Some are cancerous and aggressive.

Other primary brain tumors in adults are rare. These include:

• Ependymomas
• Craniopharyngiomas
• Pituitary tumors
• Primary lymphoma of the brain
• Pineal gland tumors
• Primary germ cell tumors of the brain

A doctor can often identify signs and symptoms that are specific to the tumor location. Some tumors may not cause symptoms until they are very large. Then they can lead to a rapid decline in the person's health. Other tumors have symptoms that develop slowly.

The specific symptoms depend on the tumor's size, location, how far it has spread, and related swelling. The most common symptoms are:

• Headaches
• Seizures (especially in older adults)
• Weakness in one part of the body
• Changes in the person's mental functions

Headaches caused by brain tumors may:

• Be worse when the person wakes up in the morning, and clear up in a few hours
• Occur during sleep
• Be accompanied by vomiting, confusion, double vision, weakness, or numbness
• Get worse with coughing or exercise, or with a change in body position

Other symptoms may include:

• Change in alertness (including sleepiness, unconsciousness, and coma)
• Changes in hearing
• Changes in taste or smell
• Changes that affect touch and the ability to feel pain, pressure, different temperatures, or other stimuli
• Clumsiness
• Confusion or memory loss
• Difficulty swallowing
• Difficulty writing or reading
• Dizziness or abnormal sensation of movement (vertigo)
• Eye abnormalities
  • Eyelid drooping
  • Pupils different sizes
  • Uncontrollable movements
• Hand tremor
• Lack of control over the bladder or bowels
• Loss of balance
• Loss of coordination
• Muscle weakness in the face, arm, or leg (usually on just one side)
• Numbness or tingling on one side of the body
• Personality, mood, behavioral, or emotional changes
• Problems with eyesight, including decreased vision, double vision, or total loss of vision
• Trouble speaking or understanding others who are speaking
• Trouble walking

Other symptoms that may occur with a pituitary tumor:

• Abnormal nipple discharge
• Absent menstruation (periods)
• Breast development in men
• Enlarged hands, feet
• Excessive body hair
• Facial changes
• Low blood pressure
• Obesity
• Sensitivity to heat or cold

Most brain tumors increase pressure within the skull and compress brain tissue because of their size and weight.

The following tests may confirm the presence of a brain tumor and identify its location:

• CT scan of the head
• EEG
• Examination of tissue removed from the tumor during surgery or CT-guided biopsy(may confirm the exact type of tumor)
• Examination of the cerebral spinal fluid (CSF) (may reveal cancerous cells)
• MRI of the head

Treatment can involve surgery, radiation therapy, and chemotherapy. Brain tumors are best treated by a team involving a neurosurgeon, radiation oncologist, oncologist, or neuro-oncologist, and other health care providers, such as neurologists and social workers.

Early treatment often improves the chance of a good outcome. Treatment, however, depends on the size and type of tumor and the general health of the patient. The goals of treatment may be to cure the tumor, relieve symptoms, and improve brain function or the person's comfort.

Surgery is often necessary for most primary brain tumors. Some tumors may be completely removed. Those that are deep inside the brain or that enter brain tissue may be debulked instead of entirely removed. Debulking is a procedure to reduce the tumor's size.

Tumors can be difficult to remove completely by surgery alone, because the tumor invades surrounding brain tissue much like roots from a plant spread through soil. When the tumor cannot be removed, surgery may still help reduce pressure and relieve symptoms.

Radiation therapy is used for certain tumors.

Chemotherapy may be used along with surgery or radiation treatment.

Other medications used to treat primary brain tumors in children may include:

• Corticosteroids, such as dexamethasone to reduce brain swelling
• Osmotic diuretics, such as urea or mannitol to reduce brain swelling and pressure
• Anticonvulsants, such as evetiracetam (Keppra) to reduce seizures
• Pain medications
• Antacids or histamine blockers to control stress ulcers

Comfort measures, safety measures, physical therapy, and occupational therapy may be needed to improve quality of life. The patient may need counseling, support groups, and similar measures to help cope with the disorder.

Patients may also consider enrolling in a clinical trial after talking with their treatment team.

Legal advice may be helpful in creating advanced directives such as a power of attorney.

For additional information, see cancer resources.

• Brain herniation (often fatal)
  • Uncal herniation
  • Foramen magnum herniation
• Loss of ability to interact or function
• Permanent, worsening, and severe loss of brain function
• Return of tumor growth
• Side effects of medications, including chemotherapy
• Side effects of radiation treatments

Call your health care provider if you develop any new, persistent headaches or other symptoms of a brain tumor.

Call your provider or go to the emergency room if you start having seizures, or suddenly develop stupor (reduced alertness), vision changes, or speech changes.

Buckner JC, Brown PD, O'Neill BP, Meyer FB, Wetmore CJ, Uhm JH. Central nervous system tumors. Mayo Clin Proc. 2007;82(10):1271-1286.

Stupp R, Roila F; ESMO Guidelines Working Group. Malignant glioma: ESMO clinical recommendations for diagnosis, treatment, and follow-up. Ann Oncol. 2009;20 Suppl 4:126-128.