Description

What is childhood visual pathway glioma?

Stage Explanation

Untreated childhood visual pathway glioma Recurrent visual pathway glioma

Treatment Option Overview

Treatment by type

Untreated Childhood Visual Pathway Glioma
Recurrent Childhood Visual Pathway Glioma
Get More Information From NCI
Changes to This Summary (04/04/2008)
About PDQ

Description

What is childhood visual pathway glioma?

Childhood visual pathway glioma is a type of brain tumor in which cancer (malignant) cells begin to grow in the tissues of the brain. The brain controls memory and learning, the senses (hearing, sight, smell, taste, and touch), and emotion. It also controls other parts of the body, including muscles, organs, and blood vessels. Other than leukemia or lymphoma, brain tumors are the most common type of cancer that occurs in children.

Gliomas are a type of astrocytoma, tumors that start in brain cells called astrocytes. A visual pathway glioma occurs along the nerve that sends messages from the eye to the brain (the optic nerve). Visual pathway gliomas are visual pathway tumors. They may grow rapidly or slowly, depending on the grade of the tumor.

The risk of developing visual pathway gliomas is increased in children with a genetic disorder called neurofibromatosis type 1 (NF-1). NF-1 is a rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin. Children with NF-1 who develop visual pathway gliomas may have a good chance of recovery and may not require treatment until symptoms appear or change.

This PDQ summary covers tumors that start in the brain (primary brain tumors). Often cancer found in the brain has started somewhere else in the body and has spread (metastasized) to the brain. This is called brain metastasis (refer to the PDQ summary on Adult Brain Tumors Treatment ¹ for more information).

Like most cancer, childhood brain tumor is best treated when it is found (diagnosed) early. If your child has symptoms, the doctor may order a computed tomographic (CT) scan, a special x-ray that uses a computer to make a picture of your child’s brain. A magnetic resonance imaging (MRI) scan, which uses magnetic waves to make a picture of your child’s brain, may also be done.

Often, surgery is required to see whether there is a brain tumor and to tell what type of tumor it is. The doctor may cut out a piece of tissue from the brain and look at it under a microscope. This is called a biopsy.

There are many types of brain tumors in children and the chance of recovery (prognosis) depends on the type of tumor, where it is located within the brain, and your child’s age and general health. See the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment ² for more information about the types of childhood brain tumors.

Stage Explanation

Once childhood visual pathway glioma is found, more tests will be done to find out the type of tumor. If a biopsy specimen is taken, the cancer cells will be looked at carefully under a microscope to see how different they are from the normal cells. This will determine the histologic grade of the tumor.

There is no staging for childhood visual pathway glioma. The treatment depends on:

• The location and size of the tumor.
• The child's age and general health.
• Whether or not the child has a condition called neurofibromatosis type 1.
• Whether or not the cancer has just been diagnosed or has recurred (come back).

Untreated childhood visual pathway glioma means that no treatment has been given except to treat symptoms.

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the brain or in the head or spinal area.

Treatment Option Overview

There are treatments for all children with visual pathway glioma. Four kinds of standard treatment are used:

• Watchful waiting (closely monitoring a patient's condition without giving any treatment until symptoms appear or change).



• Surgery (taking out the cancer in an operation).



• Radiation therapy (using high- dose x-rays to kill cancer cells).



• Chemotherapy (using drugs to kill cancer cells).

Experienced doctors working together can often give the best treatment for children with visual pathway glioma. Your child’s treatment will often be coordinated by a pediatric oncologist, a doctor who specializes in cancer in children. The pediatric oncologist may refer you to other doctors, such as a pediatric neurosurgeon (a specialist in childhood brain surgery), a pediatric neurologist, a psychologist, a radiation oncologist, and other doctors who specialize in the type of treatment your child requires.

Surgery is one treatment for visual pathway glioma. Depending on where the cancer is and the type of cancer, your child’s doctor may remove as much of the tumor as possible. If the tumor cannot be totally removed, radiation therapy and chemotherapy may also be given. If the cancer is in a place where it cannot be removed, surgery may be limited to a biopsy of the cancer.

Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation therapy for childhood brain tumors usually comes from a machine outside the body (external radiation therapy). Because radiation therapy can affect growth and brain development, clinical trials are testing ways to decrease or delay radiation therapy, especially for younger children. These include internal radiation therapy, in which radiation is put into the brain through thin plastic tubes, and hyperfractionated radiation therapy, in which radiation therapy is given in several small doses per day instead of all at once. Conformal radiation therapy uses a computer to create a 3-D picture of the tumor and the radiation beams are shaped to fit the tumor; this helps to keep the radiation away from healthy tissue as much as possible.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body. Chemotherapy is being studied to delay the use of radiation therapy in some patients. Clinical trials are studying different chemotherapy drugs for visual pathway gliomas.

Some brain tumors and cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include physical problems; changes in mood, feelings, thinking, learning or memory; and having 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 possible late effects caused by some treatments. Refer to the PDQ summary on Late Effects of Treatment for Childhood Cancer ³ for more information.

Treatment for childhood visual pathway glioma depends on the type and stage of the disease and your child’s age and overall health.

Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child go into a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have unwanted side effects. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients. Clinical trials are going on in most parts of the country for childhood visual pathway glioma. If you want more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Untreated Childhood Visual Pathway Glioma

Your child’s treatment may be one of the following:

1. For patients without symptoms or progression, observation without treatment.
2. Surgery.
3. Radiation therapy.
4. Chemotherapy.
5. Clinical trials evaluating chemotherapy to shrink the tumor and delay radiation therapy.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated childhood visual pathway glioma ⁴.

Recurrent Childhood Visual Pathway Glioma

Treatment for recurrent disease depends on the type of tumor, whether the tumor comes back in the same place or in another part of the brain, and the treatment that was given before.

If possible, the tumor may be removed during surgery. Radiation therapy may be given, especially if it was not given before. Chemotherapy may be used, and clinical trials are evaluating new chemotherapy drugs. You may wish to consider having your child treated with new methods on a clinical trial.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood visual pathway glioma ⁵.

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp® ⁶ online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

For more information from the NCI, please write to this address:

NCI Public Inquiries Office

Suite 3036A

6116 Executive Boulevard, MSC8322

Bethesda, MD 20892-8322

Search the NCI Web site

The NCI Web site ⁷ provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator ⁸. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Changes to This Summary (04/04/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site ⁷. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site ⁹. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site ⁷ or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Glossary Terms

3-dimensional. A graphic display of depth, width, and height. Also called 3-dimensional.

A procedure that uses a computer to create a 3-dimensional picture of the tumor. This allows doctors to give the highest possible dose of radiation to the tumor, while sparing the normal tissue as much as possible. Also called 3-dimensional radiation therapy.

A star-shaped cell that helps nerve cells in the brain and spinal cord work the way they should. An astrocyte is a type of glial cell.

A tumor that begins in the brain or spinal cord in small, star-shaped cells called astrocytes.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

Tissue removed from the body and examined under a microscope to determine whether disease is present.

A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.

Cancer that has spread from the original (primary) tumor to the brain.

The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (non-cancerous) or malignant (cancerous).

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

CIS. The Cancer Information Service is the National Cancer Institute's link to the public, interpreting and explaining research findings in a clear and understandable manner, and providing personalized responses to specific questions about cancer. Access the CIS by calling 1-800-4-CANCER (1-800-422-6237), or by using the LiveHelp instant-messaging service at https://cissecure.nci.nih.gov/livehelp/welcome.asp. Also called CIS.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called a clinical study.

CT scan. A series of detailed pictures of areas inside the body taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called CT scan, computerized tomography, computerized axial tomography scan, and CAT scan.

To heal or restore health; a treatment to restore health.

The process of identifying a disease by the signs and symptoms.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

The amount of medicine taken, or radiation given, at one time.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

A cancer of the brain that begins in glial cells (cells that surround and support nerve cells).

The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Grading systems are different for each type of cancer.

The study of tissues and cells under a microscope.

Radiation therapy that gives smaller doses (fractions) of radiation more often than standard radiation therapy so that the full treatment course can be given with fewer side effects. In hyperfractionation, individual doses are given more often than the standard dose of once a day. Also called hyperfractionated radiation therapy and superfractionated radiation therapy.

A type of radiation therapy in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near a tumor. Also called brachytherapy, radiation brachytherapy, and implant radiation therapy.

Side effects of cancer treatment that appear months or years after treatment has ended. Late effects include physical and mental problems and second cancers.

Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. Magnetic resonance imaging makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. Magnetic resonance imaging is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called MRI, nuclear magnetic resonance imaging, and NMRI.

To spread from one part of the body to another. When cancer cells metastasize and form secondary tumors, the cells in the metastatic tumor are like those in the original (primary) tumor.

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

The organized network of nerve tissue in the body. It includes the central nervous system (the brain and spinal cord), the peripheral nervous system (nerves that extend from the spinal cord to the rest of the body), and other nerve tissue.

NF1. A rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin. Also called NF1.

A doctor who specializes in the diagnosis and treatment of disorders of the nervous system.

A doctor who specializes in surgery on the brain, spine, and other parts of the nervous system.

Closely monitoring a patient's condition but withholding treatment until symptoms appear or change. Also called watchful waiting.

A doctor who specializes in treating cancer. Some oncologists specialize in a particular type of cancer treatment. For example, a radiation oncologist specializes in treating cancer with radiation.

The nerve that carries messages from the retina to the brain.

A part of the body that performs a specific function. For example, the heart is an organ.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

Having to do with children.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

Increase in the size of a tumor or spread of cancer in the body.

A specialist who can talk with patients and their families about emotional and personal matters, and can help them make decisions.

Energy released in the form of particles or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, and medical x-rays.

A doctor who specializes in using radiation to treat cancer.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

To become well and healthy again.

To come back or to return.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

A picture of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body.

Refers to a new primary cancer in a person with a history of cancer.

A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.

A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called standard of care or best practice.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

Treatment using substances that travel through the bloodstream, reaching and affecting cells all over the body.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A rare, slow-growing tumor of the eye.

Closely monitoring a patient's condition but withholding treatment until symptoms appear or change. Also called observation.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.