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.

Causes

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

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

Exams and Tests

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

Treatment

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.

Support Groups

For additional information, see cancer resources.

Outlook (Prognosis)

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.

Possible Complications

• 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)

When to Contact a Medical Professional

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.

Alternative Names

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