Types of glioma, neuronal, and glioneuronal tumors

Astrocytoma is the most common type of glioma diagnosed in children. It starts in a type of star-shaped glial cell called an astrocyte. Astrocytomas can form anywhere in the central nervous system.

Optic pathway glioma is a type of low-grade (slow-growing) glioma that can grow in children with a genetic condition called neurofibromatosis type 1 (NF1).

There are many types of astrocytomas, other gliomas, neuronal tumors, and glioneuronal tumors including:

• diffuse pediatric-type high-grade glioma
• infant-type hemispheric glioma
• diffuse low-grade glioma
• diffuse astrocytoma
• pilocytic astrocytoma
• high-grade astrocytoma with piloid features
• pleomorphic xanthoastrocytoma
• subependymal giant cell astrocytoma
• ganglioglioma
• desmoplastic infantile ganglioglioma/desmoplastic infantile astrocytoma
• dysembryoplastic neuroepithelial tumor

Diffuse intrinsic pontine glioma (DIPG) is a type of high-grade glioma that forms in the brain stem and most often occurs in children. To learn more, see Diffuse Intrinsic Pontine Glioma.

Ependymoma is another type of tumor that can form from glial cells, but these tumors are not treated the same as gliomas. To learn more, see Childhood Ependymoma.

Causes and risk factors for childhood glioma (including astrocytoma)

Gliomas are caused by certain changes to the way glial cells function, especially how they grow and divide into new cells. Often, the exact cause of cell changes that lead to glioma is unknown. To learn more about how cancer develops, see What Is Cancer?

A risk factor is anything that increases the chance of getting a disease. Not every child with a risk factor will develop a glioma, and it will develop in some children who don't have a known risk factor. Inherited genetic disorders that may be risk factors for glioma include:

• neurofibromatosis type 1
• tuberous sclerosis

Talk with your child's doctor if you think your child may be at risk of a glioma.

Symptoms of childhood glioma (including astrocytoma)

The symptoms of childhood gliomas depend on the following factors:

• where the tumor forms in the brain or spinal cord
• the size of the tumor
• how fast the tumor grows
• your child's age and development

Some tumors do not cause symptoms while other tumors cause symptoms based on their location in the central nervous system. It's important to check with your child's doctor if your child has any symptoms below:

• 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
• more or less energy than usual
• change in personality or behavior
• seizures
• weight loss or weight gain for no known reason
• increase in the size of the head (in infants)

These symptoms may be caused by conditions other than childhood gliomas. The only way to know is to see your child's doctor.

Tests to diagnose childhood glioma (including astrocytoma)

If your child has symptoms that suggest a central nervous system tumor such as glioma, the doctor will need to find out if they are due to cancer or another condition. The doctor will ask you when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family medical history and do a physical exam, including a neurologic exam. Depending on these results, they may recommend tests to find out if your child has a central nervous system tumor.

The following tests may be used to diagnose a glioma, neuronal tumor, or glioneuronal tumor. The results will also help you and your child's doctor plan treatment.

Magnetic resonance imaging (MRI) with or without gadolinium

MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. Sometimes 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). Magnetic resonance spectroscopy (MRS) may be done during the same MRI scan to look at the chemical makeup of the brain tissue.

Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.

Surgery to diagnose and possibly remove the glioma

Your child might have surgery to diagnose or to remove all or part of the glioma. During the surgery, the surgeon removes a part of the skull, which gives an opening to remove the tumor. Sometimes scans are done during the procedure to help the surgeon locate the tumor and remove it. A pathologist will study the tumor under a microscope. If cancer cells are found, the surgeon may remove as much tumor as safely possible during the same surgery. The piece of skull is usually put back in place after the procedure.

Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

Sometimes tumors form in a place that makes them hard to remove. If removing the tumor may cause severe physical, emotional, or learning problems, a biopsy is done and more treatment is given after the biopsy.

Children who have a rare genetic condition called NF1 may be at risk of a low-grade glioma called optic pathway glioma that forms in the area of the brain that controls vision. These children may not need a biopsy to diagnose the tumor. Surgery to remove the tumor may not be needed if the tumor does not grow and symptoms do not occur.

Getting a second opinion

You may want to get a second opinion to confirm your child's diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes or another approach, or provide more information about your child's cancer.

To learn more about choosing a doctor and getting a second opinion, see Finding Health Care Services. You can contact NCI's Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your appointments, see Questions to Ask Your Doctor about Cancer.

Stages and tumor grades of childhood glioma (including astrocytoma)

Staging is the process of learning the extent of cancer in the body and is often used to help plan treatment and make a prognosis. There is no staging system used for childhood glioma, but it is given a tumor grade. Tumor grading is based on World Health Organization (WHO) criteria.

Tumor grade describes how abnormal the cancer cells look under a microscope, how quickly the tumor is likely to grow and spread within the central nervous system, and how likely the tumor is to come back after treatment.

There are four grades of gliomas, but they are most often grouped into low grade (grades I or II) or high grade (grades III or IV).

Low-grade gliomas grow slowly and do not spread within the brain and spinal cord. But as they grow, they press on nearby healthy areas of the brain, affecting brain function. Most low-grade gliomas are treatable.

High-grade gliomas are fast growing and often spread within the brain and spinal cord, which makes them harder to treat.

Childhood gliomas usually do not spread to other parts of the body.

Recurrent glioma

When a glioma comes back after it has been treated it is called a recurrent glioma. A glioma may come back in the same place as the first tumor or in other areas of the brain or spinal cord. Tests will be done to help determine if and where the cancer has returned. The type of treatment that your child will have for recurrent glioma will depend on where it came back.

Sometimes a low-grade glioma can come back as a high-grade glioma. High-grade gliomas often come back within 3 years either in the place where the cancer first formed or somewhere else in the brain or spinal cord.

Progressive childhood glioma is cancer that continues to grow, spread, or get worse. Progressive disease can be a sign that the cancer no longer responds to treatment.

Types of treatment for childhood glioma (including astrocytoma)

There are different types of treatment for children and adolescents with glioma. You and your child's cancer care team will work together to decide treatment. Many factors will be considered, such as your child's overall health, the tumor grade, and whether the cancer is newly diagnosed or has come back.

A pediatric oncologist, a doctor who specializes in treating children with cancer, will oversee treatment. The pediatric oncologist works with other health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. Other specialists may include:

• pediatrician
• pediatric neurosurgeon
• neurologist
• neuropathologist
• neuroradiologist
• rehabilitation specialist
• radiation oncologist
• endocrinologist
• psychologist
• social worker
• child-life specialist
• fertility specialist

Your child's treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child's cancer care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.

Surgery

Surgery is used to diagnose and treat childhood gliomas, as discussed in the Tests to diagnose childhood glioma (including astrocytoma) section of this summary. After surgery, an MRI (magnetic resonance imaging) is done to see if any cancer cells remain. If cancer cells are found, further treatment depends on:

• where the remaining cancer cells are
• the grade of the tumor
• your child's age

After the doctor removes all the cancer that can be seen at the time of the surgery, some children may be given chemotherapy or radiation therapy 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.

Sometimes children with a glioma have increased fluid around the brain or spinal cord. They may need surgery to place a shunt (long, thin tube) in a ventricle (fluid-filled space) of the brain and thread it under the skin to another part of the body, usually the abdomen. The shunt carries extra fluid away from the brain so it may be absorbed elsewhere in the body. This decreases the fluid and pressure on the brain or spinal cord. This process is called a CSF shunt.

A cerebrospinal fluid (CSF) shunt (a long, thin tube) carries extra CSF away from the brain so it may be absorbed elsewhere in the body. The shunt is placed in a ventricle (fluid-filled space) in the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt has a valve that controls the flow of CSF.

Treatment of newly diagnosed childhood low-grade glioma, astrocytoma, neuronal, and glioneuronal tumors

Children with neurofibromatosis type 1 (NF1) and a central nervous system tumor, children with an optic pathway glioma, or children who had a tumor found when getting a scan for another health problem may be observed (closely watched). These children may not receive treatment until signs or symptoms appear or change or the tumor grows.

Children with tuberous sclerosis may develop low-grade tumors in the brain called subependymal giant cell astrocytoma (SEGAs). Targeted therapy with everolimus or sirolimus may be used instead of surgery, to shrink the tumors.

Children diagnosed with low-grade glioma are treated based on where the tumor is located. The first treatment is usually surgery. An MRI is done after surgery to see if any tumor remains. If the tumor was completely removed by surgery, more treatment may not be needed and the child is closely observed.

If there is tumor remaining after surgery, treatment may include:

• observation for children who had surgery to remove part of the tumor and the tumor is expected to regrow slowly
• combination chemotherapy with or without radiation therapy
• radiation therapy, which may include conformal radiation therapy, intensity-modulated radiation therapy, proton beam radiation therapy, or stereotactic radiation therapy, may be used if the tumor does not respond to chemotherapy or when the tumor begins to grow again
• a clinical trial of targeted therapy (selumetinib) with or without chemotherapy

Treatment of progressive or recurrent childhood low-grade glioma, astrocytoma, neuronal, or glioneuronal tumors

Childhood glioma, astrocytoma, glioneuronal, and neuronal tumors can be progressive or recurrent. They most often come back in the same area but can spread to other areas in the brain. Before more cancer treatment is given, imaging tests, biopsy, or surgery are done to find out if there is cancer, how much there is, and the grade.

Treatment of progressive or recurrent childhood low-grade glioma, astrocytoma, glioneuronal, and neuronal tumors may include:

• a second surgery to remove the tumor
• radiation therapy (including conformal radiation therapy), if radiation therapy was not used when the tumor was first diagnosed
• chemotherapy, if the tumor progressed or recurred where it cannot be removed by surgery
• targeted therapy (bevacizumab) with or without chemotherapy
• targeted therapy (everolimus or sirolimus)
• targeted therapy (dabrafenib or trametinib)
• a clinical trial of targeted therapy (selumetinib)

Treatment of childhood high-grade gliomas

Treatment of newly diagnosed childhood high-grade glioma may include:

• surgery to remove the tumor
• radiation therapy with or without chemotherapy
• targeted therapy
• a clinical trial of targeted therapy with a combination of dabrafenib and trametinib after radiation therapy to treat newly diagnosed high-grade glioma that has mutations in the BRAF gene
• a clinical trial of immunotherapy

Treatment of recurrent childhood high-grade gliomas

Treatment of recurrent childhood high-grade glioma may include:

• second surgery depending on tumor type, location, and length of time between treatment and recurrence
• radiation therapy
• a clinical trial of targeted therapy
• a clinical trial of immunotherapy

Prognosis and prognostic factors for childhood glioma (including astrocytoma)

If your child has been diagnosed with a glioma, you likely have questions about how serious the cancer is and your child's chances of survival. The likely outcome or course of a disease is called prognosis.

The prognosis depends on many factors, including:

• whether the tumor is a low-grade or high-grade glioma
• where the tumor has formed in the central nervous system and if it has spread within the central nervous system or to other parts of the body
• how fast the tumor is growing
• your child's age
• whether cancer cells remain after surgery
• whether there are changes in certain genes such as BRAF
• whether your child has NF1 or tuberous sclerosis
• whether your child has diencephalic syndrome, a condition which slows physical growth
• whether the glioma has just been diagnosed or has come back after treatment

Children with a low-grade glioma, astrocytoma, neuronal tumor, or glioneuronal tumor have a relatively favorable prognosis if the tumor can be removed by surgery.

Children with a high-grade glioma have a poor prognosis. Some children diagnosed with a high-grade glioma, particularly infants younger than 1 year, may have tumors with certain fusion genes. Infants with a high-grade glioma whose tumors show these genetic changes may have a better prognosis than older children with a high-grade glioma.

For glioma that has come back after treatment, prognosis and treatment depend on how much time passed between the time treatment ended and the time the glioma came back.

No two people are alike, and responses to treatment can vary greatly. Your child's cancer care team is in the best position to talk with you about your child's prognosis.

Side effects from the tumor and treatment

The signs or symptoms caused by the tumor may begin before diagnosis and continue for months or years. It is important to talk with your child's doctors about signs or symptoms caused by the tumor that may continue after treatment.

To learn more about side effects that begin during treatment for cancer, see Side Effects.

Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

• physical problems that affect:

  -

  vision, including blindness

  -

  blood vessels

  -

  hormone levels

• 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. To learn more, see Late Effects of Treatment for Childhood Cancer.

Follow-up care

As your child goes through treatment, they will have follow-up tests or checkups. Some of the tests that were done to diagnose the cancer 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.

Regular MRIs will continue to be done after treatment has ended. The results of the MRI can show if your child's condition has changed or if the glioma has come back. If the results of the MRI show a mass in the brain, a biopsy may be done to find out if it is made up of dead tumor cells or if new cancer cells are growing.

Children who received radiation therapy to treat an optic pathway glioma are at risk of developing vision changes. These changes are most likely to occur within 2 years after radiation therapy. The effect of tumor growth and treatment on the child's vision will be closely followed during and after treatment.

Coping with your child's cancer

When your child has cancer, every member of the family needs support. Taking care of yourself during this time is important. Reach out to your child's treatment team and to people in your family and community for support. To learn more, see Support for Families When a Child Has Cancer and Children with Cancer: A Guide for Parents.

Related resources

For more childhood cancer information and other general cancer resources, see:

• Pediatric Brain Tumor Consortium (PBTC)
• About Cancer
• Childhood Cancers
• CureSearch for Children's Cancer
• Adolescents and Young Adults with Cancer
• Cancer in Children and Adolescents
• For Survivors and Caregivers