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.

See also:

• Brain tumor - metastatic (cancer that has spread to the brain)
• Brain tumor - adults

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

• Located in only a small area
• Invasive (spread to nearby areas)
• Benign (not cancerous)
• 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 specific 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 non-cancerous, slow-growing cysts. They most commonly develop in children ages 5 - 8. The main treatment is surgery to remove the tumor.

Brainstem gliomas occur almost exclusively in children. The average age of development is about 6 years old. The tumor may grow very large before triggering symptoms. 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. The 5-year survival rate is low.

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). Treatment may include surgery, radiation therapy, and chemotherapy.

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

See also: Craniopharyngioma

The specific symptoms vary, but may include:

• Decerebrate posture
• Decorticate posture
• Decreased coordination -- can cause falls
• Decreased sensation of a body area
• Fever (sometimes)
• General ill feeling
• Headache -- continues and is worse when the child wakes up
• Hearing loss
• Judgment problems
• Loss of memory
• Mood wings (emotional changes)
• Nausea
• Personality and behavior changes
• Reduced alertness
• Seizures
• Speech difficulties
• Vision problems, including double vision and decreased vision
• Vomiting -- more severe in the morning
• Weakness, lethargy
• Weakness of a body area

Infants may have the following signs and symptoms:

• Bulging fontanelles
• Increased head circumference
• No red reflex in the eye
• Opisthotonos
• Separated sutures

Additional symptoms that may be associated with primary brain tumors:

• Absent menstruation
• Breathing problems
• Confusion
• Dysfunctional movement
• Eye problems, including different sized pupils, uncontrollable eye movements, and eyelid drooping
• Facial paralysis
• Hand tremor
• Hiccups
• Obesity
• Problems with the sense of smell
• Swallowing difficulty
• Tongue problems
• Uncontrollable movement

The health care provider will perform a physical exam. The child may have a positive Babinski's reflex. The physical exam can often identify signs and symptoms that are specific to the location of the tumor. However, some tumors may not cause symptoms until they are very large and cause a rapid decline in the child's mental function.

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 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 still 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 may include:

• Corticosteroids such as dexamethasone to reduce brain swelling
• Diuretics such as urea or mannitol to reduce brain swelling and pressure
• Anti-convulsants such as phenytoin 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.

• Brain herniation (often deadly)
• Permanent, progressive, profound neurological problems
• Loss of ability to interact or function
• Side effects related to chemotherapy and radiation
• Tumor returns

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. Oct 2007;82(10):1271-86.