Brain Tumors

Exploring Brain Tumors in Children

Brain tumors are the second most common type of childhood cancer, after leukemia. Following is general information about pediatric brain tumors. The type of tumor diagnosed, along with many other factors, makes each situation unique. If your child is diagnosed with a brain tumor, your medical team will provide you with more detailed, personal information.

About Pediatric Brain Tumors

The cause of most childhood brain and spinal cord tumors is unknown. The tumors may be high-grade or low-grade. High-grade tumors are likely to grow quickly and spread into other parts of the brain. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Although low-grade brain tumors grow and press on nearby areas of the brain, they rarely spread to other parts. Both types of brain tumors need to be treated.

The signs and symptoms of childhood brain and spinal cord tumors differ in every child, depending on the following:

  • Where the tumor arises — in the brain or spinal cord.
  • The size of the tumor.
  • How fast the tumor grows.
  • The child’s age and development.

A variety of tests and procedures may be used to diagnose brain and spinal cord tumors. For information about these tests, see Diagnosing Brain Tumors.

If doctors suspect a brain tumor, a biopsy may be done to remove a sample of tissue. Most childhood brain tumors are diagnosed conclusively through a biopsy or resection. The biopsy is often done through a small needle while a resection is achieved by removing part of the skull to identify and remove some or all of the tissue. A pathologist views the tissue under a microscope to look for the type of cancer cells present. This will help guide the neurosurgeon in the type of resection needed. The pathologist will also allow for a better definition of the type and grade of the brain tumor. The grade of the tumor is based on how abnormal the cancer cells look under a microscope and how many of the tumor cells are actively dividing.

Many factors affect the way a brain tumor is treated and the child’s chance of recovery, including the type of tumor, the child’s age, how fast the tumor is growing, whether cancer cells remain after surgery, if there are changes in certain genes, whether the child has other health conditions or intracranial hypertension (cerebrospinal fluid pressure within the skull is high) at the time of diagnosis, if the tumor has just been diagnosed or has recurred (come back) and where the tumor has formed and if it has spread to nearby tissue or to other parts of the body.

As you discuss treatment options with your medical team, ask about potential side effects and late effects, which are side effects that can occur months or even years after treatment ends (see Side Effects).

The Most Common Pediatric Brain Tumors

Following are common types of brain tumors diagnosed in children as well as the standard treatment options for each. Clinical trials may also be considered a treatment option for many types of brain tumors. Talk with your medical team to find out if your child may qualify for a clinical trial (see Clinical Trials).

Childhood Central Nervous System: Embryonal Tumors

Central nervous system (CNS) embryonal tumors may begin in embryonic (fetal) cells that remain in the brain after birth. CNS embryonal tumors tend to spread through the cerebrospinal fluid (CSF) to other parts of the brain and spinal cord. There are different types of CNS embryonal tumors.

Medulloblastomas, the most common CNS embryonal tumors, are fast-growing tumors that form in brain cells of the cerebellum. The cerebellum controls movement, balance and posture. The tumor can spread through the spinal fluid to other parts of the brain and spine. In rare cases, medulloblastomas can spread to the bone, bone marrow, lung or other parts of the body.

Non-medulloblastoma embryonal tumors are fast-growing tumors that usually form in brain cells outside of the cerebellum (in the cerebrum). The cerebrum controls thinking, learning, problem-solving, emotions, speech, reading, writing and voluntary movement. Non-medulloblastoma embryonal tumors may also form in the brain stem or spinal cord. There are four types of non-medulloblastoma embryonal tumors.

  • Embryonal tumors with multilayered rosettes (ETMR) are rare tumors that form in the brain and spinal cord. ETMR most commonly occur in young children and are fast-growing tumors.
  • Medulloepitheliomas are fast-growing tumors that usually form in the brain, spinal cord or nerves just outside the spinal column. They occur most often in infants and young children.
  • CNS neuroblastomas are very rare tumors that form in the nerve tissue of the cerebrum or the layers of tissue that cover the brain and spinal cord. CNS neuroblastomas may be large and spread to other parts of the brain or spinal cord.
  • CNS ganglioneuroblastomas are rare tumors that form in nerve tissue of the brain and spinal cord. They may form in one area and be fast growing or form in more than one area and be slow growing.

CNS embryonal tumors, including medulloblastoma, are typically treated with surgery, radiation therapy and/or chemotherapy. Due to the damage radiation therapy can cause to the brains of infants and young children, radiation therapy is sometimes not used for these patients.

Childhood Brain Stem Gliomas

Gliomas are tumors formed from glial cells. Glial cells in the brain hold nerve cells in place, and help protect them from disease, such as infection. Childhood brain stem gliomas are most often one of two types.

Diffuse intrinsic pontine gliomas (DIPGs) are high-grade tumors of the pons, which is located in the upper part of the brain stem and is the primary control center for many critical functions, such as breathing, heartbeat, alertness (wakefulness) and control of movement of the eyes, mouth and swallowing. DIPG is fast-growing and spreads aggressively by invading into adjacent areas of the brain and spine. It is hard to treat due to its critical location and has a poor prognosis (chance of cure). Children younger than three years diagnosed with DIPG may have a better prognosis.

Focal gliomas are slow-growing and can be found in multiple areas of the brain stem other than the pons. These are usually low-grade tumors, very responsive to therapy, and with a much better long-term prognosis.

Surgery, radiation therapy, chemotherapy, cerebrospinal fluid diversion, observation and/or targeted therapy are the standard treatments for these brain tumors.

Childhood Astrocytomas

Astrocytomas are tumors that start in star-shaped brain cells called astrocytes, a type of glial cell. Glial cells hold nerve cells in place, and help protect them from disease, such as infection. Gliomas are tumors that form from glial cells. Astrocytoma is the most common type of glioma diagnosed in children. It can form anywhere in the central nervous system (brain, brainstem and spinal cord).

Astrocytoma is typically treated with surgery and, if low-grade, observation. Radiation therapy, with or without chemotherapy is usually needed for high-grade tumors. With the discovery of specific mutations in some pediatric astrocytomas, the use of targeted therapy is now being tested. If necessary, a cerebrospinal fluid diversion procedure may also be required.

Childhood Ependymoma

Childhood ependymoma is a disease in which malignant (cancerous) cells form in the tissues of the brain and spinal cord. The brain controls vital functions such as memory and learning, emotion, and the senses (hearing, sight, smell, taste, and touch). The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body.

Ependymomas form from ependymal cells that line the ventricles and passageways in the brain and the spinal cord. The part of the brain that is affected depends on where the ependymoma forms.

Surgery and radiation therapy are the standard treatments to treat these tumors; chemotherapy may be used if the surgeon cannot remove all of the tumor at the time of diagnosis.

Childhood Central Nervous System (CNS) Germ Cell Tumors

CNS germ cell tumors are tumors that form in the brain or spinal cord. The most common places for one or more of these CNS germ cell tumors to form is near the pineal gland and/or in an area of the brain that includes the pituitary gland and the tissue just above it. There are two types of CNS germ cell tumors.

Germinomas are the most common type and generally have a good prognosis.

Nongerminomas are less common and often make certain proteins or hormones.

Three types of treatment are used, including surgery, radiation therapy and chemotherapy.

Childhood Craniopharyngiomas

Childhood craniopharyngiomas are rare ‘benign’ brain tumors found near the pituitary gland. They are usually part solid mass and part fluid-filled cyst. They are noncancerous and do not spread to other parts of the brain or to other parts of the body (although they can cause a lot of permanent damage to the parts of the brain where they arise). As they grow and press on nearby parts of the brain or other areas, they may affect hormone production (too much or too little), growth and vision.

Standard treatments for this type of brain tumor include surgery (resection), followed in some patients with focal radiation therapy.

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