Astrocytoma

What is an astrocytoma?

Childhood astrocytoma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain. Astrocytomas are tumors that start in star-shaped brain cells called astrocytes. An astrocyte is a type of glial cell. Glial cells hold nerve cells in place and help them work the way they should. There are several types of astrocytomas. They can form anywhere in the central nervous system (brain and spinal cord). Brain tumors are the third most common type of cancer in children. The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. 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. Both benign and malignant brain tumors can cause symptoms and need treatment. 

Is treatment for childhood astrocytomas different than for adults?

Brain tumors can occur in both children and adults. However, treatment for children may be different than treatment for adults. 

How do astrocytomas affect the central nervous system?

The central nervous system controls many important body functions. Astrocytomas most commonly form in these parts of the central nervous system (CNS):

Cerebrum. The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement.

Cerebellum. The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture.

Brain stem. The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.

Hypothalamus. The area in the middle of the base of the brain that controls body temperature, hunger, and thirst.

Visual pathway. The group of nerves that connect the eye with the brain.

Spinal cord. The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain for the sense of touch.

What causes childhood brain tumors and astrocytoma?

The cause of most childhood brain tumors is not known.

What are the risk factors associated with childhood brain tumors and astrocytoma?

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Parents who think their child may be at risk should discuss this with their child's doctor. Possible risk factors for astrocytoma include:

• Past radiation therapy to the brain.
• Having certain genetic disorders, such as neurofibromatosis type 1 (NF1).
  • Having NF1 may increase a child's risk of a type of tumor called visual pathway glioma. These tumors usually do not cause symptoms. Children with NF1 who develop visual pathway gliomas may not need treatment for the tumor unless symptoms, such as vision problems, appear or the tumor grows.

What are the symptoms of astrocytomas?

The symptoms of astrocytomas are not the same in every child. Symptoms are different depending on the following:

• Where the tumor forms in the brain or spinal cord
• The size of the tumor
• How fast the tumor grows
• The child's age and development

Some tumors do not cause symptoms. Other conditions may cause the same symptoms as those caused by childhood astrocytomas. Check with your child's doctor if any of the following problems occur:

• Morning headache or headache that goes away after vomiting
• Nausea and vomiting
• Vision, hearing, and speech problems
• Loss of balance and trouble walking
• Worsening handwriting or slow speech
• Weakness or change in feeling on one side of the body
• Unusual sleepiness or change in energy level
• Change in personality or behavior
• Seizures
• Weight loss or weight gain for no known reason
• Increase in the size of the head (in infants)

How will I know if my child has an astrocytoma?

Tests that examine the brain and spinal cord are used to detect (find) childhood astrocytomas. The following tests and procedures may be used:

Physical exam and history. An exam of the body to check general signs of health. This includes checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

Neurological exam. A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.

MRI (magnetic resonance imaging) with gadolinium. A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue.

CT scan (CAT scan). A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

Can childhood astrocytomas be removed?

Childhood astrocytomas are diagnosed and removed in surgery. If doctors think there may be an astrocytoma, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle to remove tissue. Sometimes, the needle is guided by a computer. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. Because it can be hard to tell the difference between types of brain tumors, you may want to have your child's tissue sample checked by a pathologist who has experience in diagnosing brain tumors. The following tests may be done on the tissue that was removed:

Immunohistochemistry study. A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer. An MIB-1 test is a type of immunohistochemistry study that checks tumor tissue for an antigen called MIB-1. This may show how fast a tumor is growing.

Light and electron microscopy. A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.

Cytogenetic analysis. A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

A biopsy may not be needed for children who have NF1. 

What affects the chance of recovery from an astrocytoma?

The prognosis (chance of recovery) and treatment options depend on the following:

• Where the astrocytoma has formed in the CNS and if it has spread
• Whether there are cancer cells left after surgery
• The grade of astrocytoma
• Whether the child has NF1
• The child’s age
• Whether the astrocytoma has just been diagnosed or has recurred (come back).
• For recurrent astrocytoma, prognosis and treatment depend on how long it was from the time treatment ended to the time the astrocytoma recurred.

What are the stages of childhood astrocytomas?

Tests are done to find out how much tumor remains after surgery and to plan further treatment. The grade of the tumor is used in place of a staging system to plan cancer treatment. Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment. There is no standard staging system for childhood astrocytoma. Treatment is based on the grade of the tumor and whether it is untreated or recurrent (has come back after treatment). The grade of the tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. The following grades are used:

Low-grade astrocytomas are slow-growing and rarely spread to other parts of the brain and spinal cord or other parts of the body. These include grade I (pilocytic, which form like a cyst and look almost like normal cells) and grade II (fibrillary, with cells that look long or slender like fibers) astrocytomas.

High-grade astrocytomas are fast-growing and often spread within the brain and spinal cord. These include grade III (anaplastic or malignant) and grade IV (glioblastoma, which spreads the fastest) astrocytomas.

Childhood astrocytomas may form at more than one place in the brain, but they do not usually spread to other parts of the body. Children who have neurofibromatosis type 1 are more likely to have tumors in more than one place. Tests are done to find out how much tumor remains after surgery and to plan further treatment. Some of the tests used to detect astrocytomas are repeated after the tumor is removed. (See the General Information section.) This is to find out how much tumor remains after surgery and to plan further treatment. An MRI (magnetic resonance imaging) is done in the first 2 days after the surgery to see if there is any tumor left. There are three ways that cancer spreads in the body:

Through tissue. Cancer invades the surrounding normal tissue.

Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.

Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer. 

What are recurrent childhood astrocytomas?

A recurrent childhood astrocytoma is an astrocytoma that has recurred (come back) after it has been treated. The cancer may come back in the same place as the first tumor or in other parts of the body. High-grade astrocytomas often recur within 3 years. There are different types of treatment for patients with childhood astrocytoma. Children with astrocytomas should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years. Some cancer treatments cause side effects months or years after treatment has ended. Six types of standard treatment are used:

• Surgery
• Cerebrospinal fluid diversion
• Watchful waiting
• Radiation therapy
• Chemotherapy
• High-dose chemotherapy with stem cell transplant

What about clinical trials?

New types of treatment are being tested in clinical trials. Patients may want to think about taking part in a clinical trial. Patients can enter clinical trials before, during, or after starting their cancer treatment. Follow-up tests may be needed. 

Learn more about our Developmental Therapeutics Phase I Clinic

What is the treatment for a child diagnosed with an astrocytoma?

Different types of treatment are available for children with astrocytomas. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with astrocytomas should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

• neurosurgeon
• neurologist
• neuropathologist
• neuroradiologist
• rehabilitation specialist
• radiation oncologist
• endocrinologist
• psychologist

Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years. Symptoms caused by the tumor may begin before diagnosis. These symptoms may continue for months or years. It is important to talk with your child's doctors about symptoms caused by the tumor that may continue after treatment. 

What are the side effects of cancer treatment?

Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

• physical problems
• changes in mood, feelings, thinking, learning, or memory
• second cancers (new types of cancer)

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. Six types of standard treatment are used:

Surgery is used to diagnose and treat childhood astrocytoma as discussed in the General Information section of this summary. If cancer cells remain after surgery, further treatment depends on:

• Where the remaining cancer cells are
• The grade of the tumor
• Age of the child

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Cerebrospinal fluid diversion is a method used to drain fluid that has built up around the brain and spinal cord. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries excess fluid away from the brain so it may be absorbed elsewhere in the body.

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Watchful waiting is often used for patients who have neurofibromatosis type1 or a tumor that is not growing and spreading.

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and location of cancer being treated. Radiation therapy to the brain can affect growth and development in young children. Certain ways of giving radiation therapy can lessen the damage to healthy brain tissue:

Conformal radiation therapy is a type of external radiation therapy. It uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to normal tissue around the tumor.

Stereotactic radiation therapy uses a rigid head frame attached to the skull to aim radiation directly to a tumor, causing less damage to normal tissue around the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing.

When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).

Combination chemotherapy is the use of more than one anticancer drug.

The way the chemotherapy is given depends on the type and location of the cancer being treated. For children younger than 3 years, chemotherapy may be given instead, to delay or reduce the need for radiation therapy.

High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

What are the new types of treatment that are being tested in clinical trials?

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. One type of targeted therapy under study for childhood astrocytomas is monoclonal antibody therapy.

Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. 

Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. 

Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. 

Patients can enter clinical trials before, during, or after starting their cancer treatment. 

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. 

Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. (See the General Information section for a list of tests.) Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the astrocytoma has recurred (come back). If the tumor recurs in the brain, a biopsy may also be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. These tests are sometimes called follow-up tests or check-ups. MRIs may be done regularly as follow-up to see if the tumor is growing back. 

Treatment options for childhood astrocytomas

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Childhood low-grade astrocytomas

When the tumor is first diagnosed, treatment for childhood low-grade astrocytoma depends on the location of the tumor and is usually surgery. An MRI is done after surgery to see if there is tumor remaining. If the tumor was completely removed by surgery, more treatment may not be needed and the child is closely watched to see if symptoms appear or change. This is also called watchful waiting. If there is tumor remaining after surgery, treatment may include the following:

• Watchful waiting
• More surgery to remove the tumor
• Cerebrospinal fluid diversion
• Radiation therapy, which may include conformal radiation therapy or stereotactic radiation therapy
• Combination chemotherapy with or without radiation therapy.
• A clinical trial of a new treatment

In some cases, children who have a visual pathway glioma will be treated by watchful waiting. In other cases, treatment may include surgery or radiation therapy. A goal of treatment is to save as much vision as possible. The effect of tumor growth on the child's vision will be closely followed during treatment. Children with neurofibromatosis type 1 (NF1) may not need treatment unless the tumor grows or symptoms, such as vision problems, appear. Children with tuberous sclerosis may develop benign (not cancer) tumors in the brain called subependymal giant cell astrocytomas (SEGAs). These tumors may be treated with drugs to shrink them instead of surgery.

Recurrent childhood low-grade astrocytomas

Before more cancer treatment is given, imaging tests, biopsy, or surgery are done to be sure cancer is present and find out how much cancer there is. Treatment of recurrent childhood low-grade astrocytoma may include the following:

• More surgery to remove the tumor
• Radiation therapy to the tumor only, if radiation therapy was not used when the tumor was first diagnosed. Conformal radiation therapy may be given
• Surgery, chemotherapy, and/or radiation therapy, if the patient had only surgery when the tumor was first diagnosed
• Chemotherapy if the tumor recurred after treatment with radiation therapy
• Chemotherapy if the tumor recurred where it cannot be removed by surgery and the patient had radiation therapy when the tumor was first diagnosed
• A clinical trial of a new treatment

Treatment of childhood high-grade astrocytoma may include the following:

• Surgery, chemotherapy, and radiation therapy to the tumor
• A clinical trial of chemotherapy with or without radiation therapy
• A clinical trial of targeted therapy with a monoclonal antibody or chemotherapy given at the same time as radiation therapy to the tumor, followed by more treatment with targeted therapy and chemotherapy

Recurrent childhood high-grade astrocytomas

Treatment of recurrent childhood high-grade astrocytoma may include the following:

• Surgery
• High-dose chemotherapy with stem cell transplant
• A clinical trial of a new treatment