Brain tumors are a mass of abnormal cells that grow out of control in various sections of the brain and spinal cord. The brain and spinal cord are collectively called the central nervous system (CNS).

Overall, tumors of the central nervous system are very rare. However, they are the second leading cause of cancer-related deaths in children (after leukemia), according to the American Cancer Society (ACS). Childhood brain tumors account for 17 percent of malignant childhood cancers. The number of children diagnosed with brain tumors has not changed significantly in recent years.

Most brain tumors in children are primary, meaning the cancer originated in the brain rather than spread to the brain from cancer elsewhere in the body. There are many types of brain tumors, which can be benign (noncancerous) or malignant (cancerous). Although benign tumors are often not life threatening in other parts of the body, they can be serious when they occur in the brain. Brain tumors cause symptoms due to increased pressure on the brain and the inability of the tissues to expand in the rigid skull. Depending on which type of tissue is affected, various problems can develop with vital functions.

Brain tumors rarely spread outside the central nervous system but can spread within the brain and spinal cord. Symptoms vary depending on the type and location of the tumors, as well as any metastasis (spread).

Recurring headaches with or without nausea and vomiting, vision and hearing problems and loss of balance may indicate the presence of a brain tumor. However, these symptoms may be related to less serious medical conditions. Parents should consult with a physician if their child develops these symptoms to determine the cause.

The causes of brain tumors in children are not known. There is very little evidence of environmental or genetic relationship to the development of this type of cancer. However, a very small percentage of brain tumors are related to inherited factors. There is no way to screen for or prevent brain tumors in children, because their cause has not been identified. In addition, risk factors cannot be isolated for brain tumors. For these reasons, there is no known prevention for brain tumors in children.

Early diagnosis is crucial to a good outcome. A biopsy will confirm the presence of a tumor as well as the type and grade of the tumor. The major tests used to diagnose a brain tumor include various imaging tests and possibly lumbar puncture (collection of spinal column cerebral fluid).

A cancer care team ensures that all aspects of the child’s medical and surgical needs are considered in the treatment process. For brain tumors in a child, this team typically includes a pediatric medical oncologist (who specializes in cancer in children), a neurosurgeon and other healthcare professionals who diagnose and treat cancer. Standard treatment options are a combination of surgical removal of the tumor, chemotherapy and/or radiation therapy. Treatment depends on the type and location of the cancer, the grade of the tumor and the general health of the child.

A child’s chance of survival with brain cancer varies greatly with the type and location of the brain tumor. More than half of the children diagnosed with brain tumors survive longer than five years, according to the ACS. This statistic includes all types of brain tumors and some forms have a better survival rate.

Brain tumors are a collection of abnormal cells that multiply rapidly in the brain or spinal cord. They are the third most common type of cancer in children (besides leukemia and lymphoma) according to the National Cancer Institute (NCI). Brain tumors are the second leading cause of cancer-related deaths in children according to the American Cancer Society (ACS), after leukemia.

Brain tumors in children are usually primary, meaning they originate in the central nervous system. The central nervous system is composed of the brain, spinal column, optic and cranial nerves. There are three categories of tumors that can affect the central nervous system. These include:

• Infratentorial tumors. Tumors located in the cerebellum or the fourth ventricle, which lies in the back of the brain just above the brain stem. This part of the brain regulates balance, coordination, posture and walking. About 50 percent of brain tumors in children are infratentorial, according to the NCI.
  
  
• Supratentorial tumors. Tumors that occur in the other regions of the brain that do not include the cerebellum or the brain stem. This is the largest area at the top of the brain that includes the cerebrum. This area controls emotions, behavior, speech and languard, cognition and the basic senses (e.g., hearing, smell).
  
  
• Spinal cord tumors. Tumors that occur in the spinal cord, the large bundle of nerve fibers connected to the brain that sends and receives signals to and from the brain.

Childhood brain tumors are diverse because there are many types of tissue in the brain that can be affected. Brain tumors rarely metastasize (spread) to other parts of the body, but as they grow, they can impinge upon surrounding brain tissue. This affects functions controlled by the brain and causes damage that can be fatal.

Malignant brain tumors in children are nearly always primary cancers, meaning that they originated within the brain and did not spread from another body area. Prognosis (chance of recovery) of children with brain tumors is determined by:

• Age of patient
• Type of brain tumor
• Grade or level of aggressiveness or growth
• Location of the tumor
• Amount of tumor removed
• Metastasis or spread of tumor (rarely outside of the central nervous system)
• Underlying health conditions
• Recurrences

The ACS estimates that 3,200 tumors of the central nervous system are diagnosed each year in children under the age of 20. Of these tumors, about one-fourth are considered benign (noncancerous) growths.

Staging is the process of determining how widespread a cancer is in a patient. Currently, the American Joint Committee on Cancer (AJCC) has not approved an official staging system for malignant brain and spinal cord tumors. However, the location, cell type and grade of the tumor (how fast it is growing) can be used to determine appropriate treatments and the prognosis for recovery. Overall, more than one-half of the patients with childhood brain tumors survive longer than five years, according to the ACS. However, this statistic is based on all types of brain tumors. Some brain tumors, such as astrocytomas, can be cured with surgery in a high percentage of patients.

Most childhood brain tumors are primary, meaning they originate in the brain and have not spread from cancer located elsewhere in the body. There are three regions of the brain and spinal cord that contain cells that may develop into childhood brain tumors. The tumors are categorized by where they are located in the brain and the cell type. The following are regions of the brain where tumors grow:

• Infratentorial tumors represent 50 percent of childhood brain tumors. The location of these cells is largely in the cerebellum (the posterior portion of the brain located at the back of the neck where the spinal column begins) and the fourth ventricle (cavity located near the cerebellum).
  
  
• Supratentorial tumors are located in the rest of the regions of the brain that are not in the cerebellum, brain stem or spinal cord.
  
  
• Spinal cord tumors account for 1 to 2 percent of childhood nervous system tumors. They are rare benign or malignant tumors in the spinal cord (nerve bundles connected to the brain). These tumors can compress the spinal column and cause pain or numbness in the spine, arms, legs or muscles of the body. Additional problems might also occur depending on the location of the tumor and the nerves it affects.

Gliomas begin in glial (supportive tissue) cells and are the most common primary brain tumors. Gliomas found in children include:

• Astrocytoma. Glial tumors that come from connective tissue in the brain. They can be malignant or benign and can be located anywhere. They are classified into high- and low-grade categories. Astrocytomas are the most common brain tumor type in children and usually occur in the cerebellum (cerebellar astrocytomas).
  
  
• Brain stem glioma. Malignant or benign tumors that develop in the brain stem, which is connected to the spinal cord. The brain stem is at the back of the neck at the base of the brain. These tumors occur almost exclusively in children and cannot be surgically removed.
  
  
• Ependymoma. Slow-growing, malignant tumors that develop in the ependyma, cells that line the ventricles (cavities) of the brain and spinal cord. Ependymal tumors obstruct the cerebrospinal fluid in the brain and spinal cord, causing brain pressure and pain. This can affect memory, learning, the five senses (hearing, touch, sight, smell, taste) and emotions. These tumors may spread to other parts of the body through the spinal fluid. One in 10 childhood brain tumors are ependymomas, according to the National Cancer Institute (NCI).
  
  
• Optic nerve glioma. Gliomas that are located in the optic nerves. These tumors can lead to blindness and often are associated with neurofibromatosis, a neural disease present from birth. These tumors cannot be surgically removed and are difficult to treat.
  
  
• Oligodendrogliomas. Tumors that begin in oligodendrocytes (a type of glial cell). These are usually found in the cerebrum. They occur most often in middle-aged adults and rarely in children.

The following brain tumors do not begin in glial cells:

• Medulloblastoma. These tumors are also called primitive neuroectodermal tumor (PNET). They are malignant or benign tumors that develop from immature nerve cells usually located in the cerebellum. This form of brain tumor is more common in boys than girls. Medulloblastomas cause brain pressure and can spread to the spinal cord. One in five childhood brain tumors are medulloblastomas, according to the NCI.
  
  
• Craniopharyngioma. Benign tumors that occur above the pituitary gland, located at the base of the brain. The pituitary is the size of a pea and controls hormonal and vital body functions. Tumors in this area can cause problems with pituitary and hypothalamic functions affecting hormone balance. Craniopharyngiomas do not spread but impinge upon the structures around them. Associated imbalances of hormones can cause short stature and several other problems. These tumors typically develop before age 20.
  
  
• Germ cell tumor. Embryonic sex cells that migrate to the brain instead of the ovaries or testicles can produce germ cell tumors. These tumors include: germinomas (most frequently occurring germ cell tumor type), embryonal cell carcinomas, choriocarcinomas and teratomas. The cells can travel from the center of the brain to other parts of the brain and spinal cord.
  
  
• Pineal region tumors. There are two types of tumors that can develop in this region. Pineocytomas are slow growing. Pineoblastomas are fast growing and form in the pineal gland, which is a tiny organ that produces melatonin (a hormone that controls sleep cycles).
  
  
• Pituitary tumors. Benign tumors in the pituitary, located at the base of the brain. It produces hormones that control the glands in the body.
  
  
• Schwannoma. Benign tumors that arise in the nerve sheath (covering). These tumors are rare in children.
  
  
• Meningiomas. Benign tumors that occur in the outer brain covering just beneath the skull bones. These occur most often in middle-aged adults rather than children.
  
  
• Ganglioglioma. Tumor containing mature neurons and glial cells.
  
  
• Choroid plexus tumors. Tumors in the choroid plexus are located in the ventricles of the brain. They can be either benign or malignant.
  
  
• Chordoma. Tumors located in the bones of the skull or spinal cord at the back of the neck. They often recur over 10 to 20 years, but do not spread to other organs.

The causes of brain tumors in children are not fully understood. Although some of the chemical changes and alterations to the genetic material of DNA can be isolated and observed, not enough information can be gathered or confirmed at this time to determine causes. No reliable associations exist showing a cause and effect related to genetic or environmental causes in most cases.

A family history involving multiple tumor development and childhood brain tumors indicate a genetic risk factor in some rare cases. Generally, familial tumors are marked by multiple tumor locations and occurrence at younger ages. According to the American Cancer Society (ACS), these family history factors include the following conditions:

• Neurofibromatosis type 1 (von Recklinghausen’s disease). The gene responsible can be identified and is the most common source of familial brain and spinal cord tumors.
  
  
• Neurofibromatosis type 2. The gene responsible is known and is a less common source. It is responsible for tumors in the acoustic (hearing) nerves, and meningiomas or spinal cord ependymomas.
  
  
• Tuberous sclerosis. Involves astrocytomas and includes benign tumors located in the skin, heart or kidneys.
  

  

• Von Hippel-Lindau disease. Associated with blood vessel tumors of the cerebellum or retina, and kidney cancer.
  
  
• Li-Fraumeni syndrome. Increases the risk of breast cancer, soft tissue sarcomas, leukemia, adrenal gland cancers and gliomas.

Signs and symptoms depend on the size, location, rate of growth and cell type of the tumor as well as the child’s age. Childhood brain tumors are diverse because there are many types of tissue in the brain that can be affected. Similarly, the symptoms that manifest from childhood brain tumors can also be diverse. Common symptoms include:

• Recurring and persistent headaches
• Loss of balance and difficulty walking
• Problems with writing or speaking
• Vision and hearing problems
• Nausea and/or vomiting
• Sleepiness and tiredness (fatigue)
• Changes in personality or behavior
• Unexplained weight loss or gain
• Changes in feeling or muscle strength, especially on one side of the body
• Cognitive problems (e.g., thinking, memory)
• Hormonal disorders
• Seizures

The above can be found in many other less serious conditions. Parents should consult with a physician who can perform tests to diagnose the potential cause of the symptoms.

Early diagnosis of brain tumors in children is crucial for a good outcome. The following methods may be used by a physician to determine the presence of brain tumors in children who have suspicious symptoms:

• Medical history. A personal medical history of the child as well as a family medical history.
  
  
• Physical examination. An examination of the patient for signs of disease. The child is examined for tumor masses and lumps and given a neurological examination to detect problems with eye movement, balance, strength, sensation, coordination or other factors.
  
  
• Blood tests, including blood chemistry and a complete blood count (CBC). These tests are taken to determine chemical or hormonal imbalances that may indicate cancer cells in the tissues or organs.
  
  
• Genetic testing. DNA can be checked for the presence of oncogenes and deletions of tumor suppressor genes. Genetic alterations such as these can indicate genetic predisposition for cancer as well as potential prognosis for survival. Physicians may also test for any inherited attributes indicating a risk for brain tumors in children.

Imaging tests may be used to determine tumor location, size and other factors. They may also be used following diagnosis to guide treatments. Imaging tests used for brain tumors in children may include:

• CAT scan. Images are taken of the body from different angles to locate tumors using specialized x–ray equipment. In some patients, a contrast medium (dye) may be used to highlight areas of the body. Tumors in the brain, spinal cord and other areas may be detected with a CAT scan.

• Magnetic resonance imaging (MRI). Magnetic and radio waves are used to create cross-sectional detailed images of internal organs, including the brain and spinal cord to locate tumors.
  

• Positron emission tomography (PET) scan. A radioactive sugar is injected into the bloodstream to detect the location of cancer in the body. Cancer cells absorb the sugar substance and appear as hot spots on the images. PET scans are often used to determine if, and where, cancer has spread.
  
  
• Bone scan. Radioactive material injected into the bloodstream collects in the bones and images are taken by a scanner. The images detect cancer cells in the bones, a possible location of cancer metastasis (spread), although this is uncommon among children with brain tumors.
  
  
• Angiogram. Dye is injected into the bloodstream to show blood vessels in and around a tumor.
  
  
• X-rays. The use of radiation to create images on film of organs in the body (used less often with brain tumors).
  
  
• Magnetic resonance spectroscopy (MRS). Magnetic scanner used to measure the metabolism in the brain. This is rarely used in diagnosis of brain cancers, but may be used to guide treatments.
  
  
• Single-photon emission computerized tomography (SPECT). Imaging technique that measures blood in the brain. This is more often used to determine treatment options for brain cancers, rather than for diagnosis.

After a tumor has been detected, a biopsy is needed to determine if it is malignant or benign. A standard biopsy involves removing a sample of tissues from the brain, spinal cord and/or lymph nodes for analysis under a microscope by a pathologist. Infrequently, bone marrow biopsy may also be performed to determine if cancer has spread beyond the central nervous system. This involves removing a sample of bone marrow for analysis. While most brain cancers do not spread, medulloblastomas can spread beyond the nervous system.

A biopsy will reveal the type of cells involved and the extent of abnormality of the cells which determines the grade of the cancer. Higher-grade cells are more abnormal, grow faster and are therefore more malignant than lower grade cells. There is currently no official staging system for malignant brain and spinal cord tumors. Therefore, the grade and cell type of the cancer are used to determine appropriate treatments and prognosis for recovery.

Treatment is planned according to the type and grade of the tumor, its location in the brain and the child’s age and overall health. Standard treatment and clinical trial options are available. As with adults, treatment of brain tumors in children requires the involvement of a cancer care team. Some of the healthcare professionals who may be involved include:

• Pediatric medical oncologist (physician who specializes in cancer in children)
  
  
• Pediatric neurosurgeon (brain and spinal cord surgeon for children)
  
  
• Pediatric neurologist
  
  
• Radiation oncologist
  
  
• Neuropathologist
  
  
• Endocrinologist
  
  
• Rehabilitation specialists
  
  
• Psychologist

Standard treatment options for childhood brain tumors include:

• Surgery. Removal of a part or all of cancerous cell growth. Optimally, the entire tumor is removed. Surgery is not possible with all types of brain tumors, particularly if there is metastasis or if there is danger in damaging surrounding tissue. Risks include bleeding and damage to blood vessels and nerves.
  
  
• Chemotherapy. The use of powerful drugs to destroy cancer cells. It may be administered before and/or after surgery, when the whole tumor cannot be removed or when there is metastasis to other areas.
  
  
• Radiation therapy. Use of high-energy x-ray beams to kill or shrink cancerous cells. If cancer cannot be removed, radiation may be used as a form of treatment. Internal radiation involves placing radioactive “seeds” directly in the area of the cancer. External radiation involves the use a machine outside the body that directs the precise radiation beams to the site of the tumor. The type of radiation given depends on the type and stage of the brain tumor. Radiation is usually not given to children under three years because it can affect growth and development of brain tissue.

According to the American Cancer Society (ACS), survival rates of some of the most common childhood brain tumors are:

According to the National Cancer Institute (NCI), more than half of children diagnosed with brain tumors will survive five years from diagnosis. Some brain tumors have an even better prognosis. According to the American Cancer Society (ACS), 90 percent of astrocytomas of the cerebellum are cured by surgery. The outlook for recovery varies based on multiple factors.

According to the American Cancer Society, the following areas of research and experimentation are in progress to improve treatments for brain tumors in children:

• Primary chemotherapy (neo-adjuvant chemotherapy). Chemotherapy is being used in children and infants right after surgery to delay or decrease the use of radiation. One study indicated that children with ependyomas who were given prolonged chemotherapy did not need radiation therapy.
  
  
• Stereotactic radiosurgery. Concentrated radiation and/or photon beams are directed at the cancerous cells, reducing damage to surrounding healthy tissues. This treatment may be beneficial for children who have brain or spinal cord tumors that are small and in locations that are not operable.
  
  
• Intensity modulated radiation therapy (IMRT). A variation of stereotactic radiosurgery, this technique also employs precise application of radiation to specific cancer cells.
  
  
• Gene therapy. High-grade gliomas are treated with a virus containing genetic DNA that weakens tumor cells to become susceptible to antivirus drugs. These drugs kill the cancer cells but are harmless to the healthy cells.
  
  
• Newer neurosurgical techniques. Computer-guided surgery, magnetic resonance imaging (MRI) and cortical mapping are being developed for use during surgical procedures to aid in precision.
  
  
• High-dose chemotherapy. An experimental, radical procedure for brain tumors in children that extracts healthy bone marrow from the patient and then applies very high doses of chemotherapy. Finally, the healthy bone marrow is placed back into the patient. This highly toxic treatment has yielded good results in clinical trials but is not yet available as a standard form of treatment for brain tumors.
  
  
• Growth factor inhibitors. Drugs that make growth factor produced by tumors ineffective for supporting their growth.
  
  
• Angiogenesis inhibitors. Drugs used to prevent the formation of blood vessels, which promote the growth of tumors.
  
  
• Hypoxic cell sensitizers (radiosensitizers). Drugs used to increase sensitivity to radiation in tumor cells.
  
  
• Immunotherapy (biological therapy). Drugs are used to stimulate the body’s immune system to fight tumor cells as invaders. Monoclonal antibodies and immune modulators, such as interferon, are being investigated as a treatment.
  
  
• Specialized drugs. Agents that can help combat the “blood-brain barrier” are being investigated to help chemotherapy drugs reach the cancer. Some agents are placed directly into the tumor in the form of wafers for slow-release of the anti-cancer drug. In other studies, researchers are examining the use of small tubes inserted into the brain to allow drugs to pass into the tumors.

Many of the newer treatments are only available through clinical trials. The child’s oncologists can best determine if a patient is a candidate for a clinical study. Parents should discuss this option with their child’s physicians.

Preparing questions in advance can help patients have more meaningful discussions with their physicians regarding their conditions. Parents may wish to ask their doctor the following questions about brain tumors in their child:

1. What tests will be used to diagnose my child’s brain tumor?
   
   
2. What type of brain tumor does my child have?
   
   
3. Where is the tumor located?
   
   
4. What is the grade of the tumor?
   
   
5. Has the cancer spread outside of the brain?
   
   
6. Is my child a candidate for surgery? If so, what will be involved?
   
   
7. Will other treatments be necessary either before or after surgery?
   
   
8. What are the risks associated with the treatments?
   
   
9. What can I expect in terms of recovery?
   
   
10. What is the expected prognosis for my child?
    
    
11. What can I do to help prepare my child for treatment?
    
    
12. What are the chances the cancer will return?
    
    
13. How will my child be monitored after treatment?
    
    
14. Can your recommend a support group for us?