21st Century Pediatric Cancer Sourcebook: Childhood Astrocytomas, Oligodendrogliomas, Oligoastrocytomas, Glioblastoma Multiforme - Clinical Data, Practical Information for Patients, Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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This material represents a snapshot in time, with authoritative information formatted for ebook reading that was up-to-date at the moment of publication. For the latest cancer updates, please be sure to visit the NCI website:
From our Guide to Leading Medical Websites, here are three valuable sites with authoritative cancer information:
OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
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PART ONE
Chapter 1A: Childhood Astrocytomas
PART TWO
Chapter 1B Late Effects of Treatment for Childhood Cancer - Patient Version
Chapter 2B: Pediatric Supportive Care
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: When Someone You Love Is Being Treated For Cancer
Chapter 15B: Living Beyond Cancer: Finding a New Balance
Chapter 16B: Caring for the Caregiver
Chapter 17B: Young People With Cancer, A Handbook For Parents
Chapter 18B: When Cancer Returns
Chapter 19B: When Someone You Love Has Advanced Cancer / Support for Caregivers
Chapter 20B: Chemotherapy and You
Chapter 21B: Managing Chemotherapy Side Effects - Anemia
Chapter 22B: Managing Chemotherapy Side Effects - Appetite Changes
Chapter 23B: Managing Chemotherapy Side Effects - Bleeding Problems
Chapter 24B: Managing Chemotherapy Side Effects - Constipation
Chapter 25B: Managing Chemotherapy Side Effects - Memory Changes
Chapter 26B: Managing Chemotherapy Side Effects - Mouth and Throat Changes
Chapter 27B: Managing Chemotherapy Side Effects - Nerve Changes
Chapter 28B: Managing Chemotherapy Side Effects - Pain
Chapter 29B: Managing Chemotherapy Side Effects - Skin and Nail Changes
Chapter 30B: Managing Chemotherapy Side Effects - Swelling (Fluid retention)
Chapter 31B: Targeted Cancer Therapies
Chapter 33B : Follow-up Care After Cancer Treatment
Chapter 34B: Radiation Therapy and You
Chapter 36B: Understanding Radiation Therapy, What To Know About External Beam Radiation Therapy
Chapter 37B: Radiation Therapy for Cancer
Chapter 38B: Managing Radiation Therapy Side Effects - What To Do When Your Mouth or Throat Hurts
Chapter 39B: What To Do About Hair Loss (Alopecia)
Chapter 42B: Managing Radiation Therapy Side Effects - Changes When You Urinate
Chapter 43B: Managing Radiation Therapy Side Effects What To Do About Mild Skin Changes
Chapter 45B: General Cancer Information And Resources
Chapter 46B: Cancer And The Environment - What You Need to Know, What You Can Do
Chapter 48B: FDA Warning: Beware of Online Cancer Fraud
Chapter 49B: FDA Office of Oncology Drug Products
Chapter 50B: Understanding the HIPAA Privacy Rule
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Childhood Astrocytomas Treatment - Last Modified: 01/06/2011
General Information About Childhood Astrocytomas * Stages of Childhood Astrocytomas * Recurrent Childhood Astrocytomas * Treatment Option Overview * Treatment Options for Childhood Astrocytomas Untreated Childhood Astrocytomas * Recurrent Childhood Astrocytomas * To Learn More About Childhood Astrocytomas
Childhood astrocytoma is a disease in which benign (non-cancer) or malignant (cancer) cells form in the tissues of the brain.
The central nervous system controls many important body functions.
The cause of most childhood brain tumors is not known.
The symptoms of astrocytomas are not the same in every child.
Tests that examine the brain and spinal cord are used to detect (find) childhood astrocytomas.
Childhood astrocytomas are diagnosed and removed in surgery.
Certain factors affect prognosis (chance of recovery) and treatment options.
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.
This summary is about the treatment of primary brain tumors that begin in the glial cells in the brain. Information is included about the following tumors that form from glial cells:
Astrocytomas. * Oligodendrogliomas. * Oligoastrocytomas. * Glioblastoma multiforme.
Treatment of metastatic brain tumors is not discussed in this summary. Metastatic brain tumors are formed by cancer cells that begin in other parts of the body and spread to the brain.
Brain tumors can occur in both children and adults. However, treatment for children may be different than treatment for adults.
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.
The cause of most childhood brain tumors is not known.
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.
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).
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.
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.
Certain factors affect prognosis (chance of recovery) and treatment options.
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.
Stages of Childhood Astrocytomas
Key Points for This Section
The grade of the tumor is used in place of a staging system to plan cancer treatment.
Low-grade astrocytomas
High-grade astrocytomas
Tests are done to find out how much tumor remains after surgery and to plan further treatment.
There are three ways that cancer spreads in the body.
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
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
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 3 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.
The three ways that cancer spreads in the body are:
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.
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.
Treatment Option Overview
Key Points for This Section
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
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.
There are different types of treatment for patients with childhood 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:
Pediatric 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.
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
Surgery is used to diagnose and treat childhood astrocytoma as discussed in the General Information 5 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
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
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
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.
For children younger than 3 years, chemotherapy may be given instead, to delay or reduce the need for radiation therapy.
Chemotherapy
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.
High-dose chemotherapy with stem cell transplant
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.
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.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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 3 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.
Untreated Childhood Astrocytomas
Untreated low-grade astrocytoma
When the tumor is first diagnosed, treatment for low-grade childhood 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 conformal radiation therapy directed to the tumor only.
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.
Untreated high-grade astrocytoma
Treatment of untreated high-grade childhood astrocytoma may include the following:
Surgery, chemotherapy, and radiation therapy to part or all of the brain.
A clinical trial of chemotherapy with or without radiation therapy.
A clinical trial of surgery followed by chemotherapy given at the same time as radiation therapy, followed by combination chemotherapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood cerebellar astrocytoma, untreated childhood cerebral astrocytoma, untreated childhood brain stem glioma, untreated childhood visual pathway and hypothalamic glioma and untreated childhood subependymal giant cell astrocytoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Recurrent Childhood 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.
Recurrent low-grade astrocytoma
Treatment of recurrent low-grade childhood 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.
Recurrent high-grade astrocytoma
Treatment of recurrent high-grade childhood astrocytoma may include the following:
Surgery and/or chemotherapy.
High-dose chemotherapy with stem cell transplant.
A clinical trial of a new treatment.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood cerebellar astrocytoma 12, recurrent childhood cerebral astrocytoma 13, recurrent childhood brain stem glioma 14, recurrent childhood visual pathway and hypothalamic glioma 15 and recurrent childhood subependymal giant cell astrocytoma 16. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 11.
Glossary Terms
antibody (AN-tee-BAH-dee)
A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.
astrocyte (AS-troh-site)
A large, star-shaped cell that holds nerve cells in place and helps them develop and work the way they should. An astrocyte is a type of glial cell.
astrocytoma (AS-troh-sy-TOH-muh)
A tumor that begins in the brain or spinal cord in small, star-shaped cells called astrocytes.
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
biopsy (BY-op-see)
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.
brain stem
The part of the brain that is connected to the spinal cord.
brain tumor
The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (not cancer) or malignant (cancer).
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a 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. Also called malignancy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
central nervous system (SEN-trul NER-vus SIS-tem)
The brain and spinal cord. Also called CNS.
cerebellum (ser-uh-BEL-um)
The portion of the brain in the back of the head between the cerebrum and the brain stem. The cerebellum controls balance for walking and standing, and other complex motor functions.
cerebrum (seh-REE-brum)
The largest part of the brain. It is divided into two hemispheres, or halves, called the cerebral hemispheres. Areas within the cerebrum control muscle functions and also control speech, thought, emotions, reading, writing, and learning.
chemical (KEH-mih-kul)
A substance made up of elements, such as hydrogen or sodium.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
contrast material
A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.
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 CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
disorder (dis-OR-der)
In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.
electron microscope (ee-LEK-tron MY-kroh-SKOPE)
A microscope (device used to magnify small objects) that uses electrons (instead of light) to produce an enlarged image. An electron microscope shows tiny details better than any other type of microscope.
gadolinium (GA-duh-LIH-nee-um)
A metal element that is used in magnetic resonance imaging (MRI) and other imaging methods. It is a contrast agent, which helps show abnormal tissue in the body during imaging with a special machine.
genetic (jeh-NEH-tik)
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
glial cell (GLEE-ul sel)
Any of the cells that hold nerve cells in place and help them work the way they should. The types of glial cells include oligodendrocytes, astrocytes, microglia, and ependymal cells. Also called neuroglia.
glioblastoma multiforme (GLEE-oh-blas-TOH-muh MUL-tih-form)
A fast-growing type of central nervous system tumor that forms from glial (supportive) tissue of the brain and spinal cord and has cells that look very different from normal cells. Glioblastoma multiforme usually occurs in adults and affects the brain more often than the spinal cord. Also called GBM, glioblastoma, and grade IV astrocytoma.
grade
A description of a tumor based 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.
immunohistochemistry (IH-myoo-noh-HIS-toh-KEH-mih-stree)
A technique used to identify specific molecules in different kinds of tissue. The tissue is treated with antibodies that bind the specific molecule. These are made visible under a microscope by using a color reaction, a radioisotope, colloidal gold, or a fluorescent dye. Immunohistochemistry is used to help diagnose diseases, such as cancer, and to detect the presence of microorganisms. It is also used in basic research to understand how cells grow and differentiate (become more specialized).
injection
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
laboratory test (LA-bruh-tor-ee...)
A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.
light microscope (lite MY-kroh-SKOPE)
A microscope (device to magnify small objects) in which objects are lit directly by white light.
magnetic resonance spectroscopic imaging (mag-NEH-tik REH-zoh-nants SPEK-troh-SKAH-pik IH-muh-jing)
A noninvasive imaging method that provides information about cellular activity (metabolic information). It is used along with magnetic resonance imaging (MRI) which provides information about the shape and size of the tumor (spatial information). Also called 1H-nuclear magnetic resonance spectroscopic imaging, MRSI, and proton magnetic resonance spectroscopic imaging.
malignant (muh-LIG-nunt)
Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
membrane (MEM-brayn)
A very thin layer of tissue that covers a surface.
metastatic (meh-tuh-STA-tik)
Having to do with metastasis, which is the spread of cancer from the primary site (place where it started) to other places in the body.
microscope (MY-kroh-SKOPE)
An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.
MRI
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. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
nausea
A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.
nerve
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.
nerve cell
A type of cell that receives and sends messages from the body to the brain and back to the body. The messages are sent by a weak electrical current. Also called neuron.
neurofibromatosis type 1 (NOOR-oh-FY-broh-muh-TOH-sis tipe 1)
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.
neurological exam (NOOR-oh-LAH-jih-kul eg-ZAM)
A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.
oligoastrocytoma (AH-lih-goh-AS-troh-sy-TOH-muh)
A brain tumor that forms from both oligodendrocytes and astrocytes, which are types of glial cells (cells that cover and protect nerve cells in the brain and spinal cord and help them work the way they should). An oligoastrocytoma is a type of mixed glioma.
oligodendroglioma (AH-lih-goh-DEN-droh-glee-OH-muh)
A rare, slow-growing tumor that begins in oligodendrocytes (cells that cover and protect nerve cells in the brain and spinal cord). Also called oligodendroglial tumor.
organ
A part of the body that performs a specific function. For example, the heart is an organ.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
primary tumor (PRY-mayr-ee TOO-mer)
The original tumor.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee)
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 irradiation and radiotherapy.
radioisotope (RAY-dee-oh-I-suh-tope)
An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.
recover (ree-KUH-ver)
To become well and healthy again.
recur
To come back or to return.
recurrent cancer (ree-KER-ent KAN-ser)
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.
risk factor (... FAK-ter)
Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.
seizure (SEE-zhur)
Convulsion; a sudden, involuntary movement of the muscles.
spinal cord (SPY-nul kord)
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.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
tissue (TISH-oo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
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 cancer), or malignant (cancer). Also called neoplasm.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
visual pathway glioma (VIH-zhoo-ul ... glee-OH-muh)
A rare, slow-growing tumor that usually forms in the optic nerve, optic chiasm, or optic tract. These are parts of the nervous system that carry messages from the eye to the brain. Also called optic pathway glioma.
vomit
To eject some or all of the contents of the stomach through the mouth.
x-ray
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.
* * * * * * * * * * * *
Childhood Astrocytomas Treatment - Last Modified: 11/19/2010
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2002, childhood cancer mortality has decreased by more than 50%.[3] Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment.
Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.
Clinicopathologic Classification of Childhood Astrocytomas and Other Tumors of Glial Origin
The pathologic classification of pediatric brain tumors is a specialized area that is undergoing evolution; review of the diagnostic tissue by a neuropathologist who has particular expertise in this area is strongly recommended.
Childhood astrocytomas and other tumors of glial origin are classified according to clinicopathologic and histologic subtype and are histologically graded from grade I to IV according to the World Health Organization’s (WHO) histologic typing of central nervous system (CNS) tumors.[1] Tumor types are based on the glial cell type of origin: astrocytomas (astrocytes), oligodendroglial tumors (oligodendrocytes), mixed gliomas (cell types of origin include oligodendrocytes, astrocytes, and ependymal cells) and neuronal tumors (with or without an astrocytic component).
WHO histologic grades are commonly referred to as low-grade gliomas or high-grade gliomas.
Table 1. WHO Histologic Grade and Corresponding Classification for Tumors of the Central Nervous System
WHO Histologic Grade Grade Classification
I Low-grade
II Low-grade
III High-grade
IV High-grade
In 2007, the WHO further categorized astrocytomas, oligodendroglial tumors, and mixed gliomas according to histopathologic features and biologic behavior. In 2004, it was determined that the pilomyxoid variant of pilocytic astrocytoma may be a more aggressive variant and may be more likely to disseminate, and it was reclassified by the WHO as a grade II tumor.[1,2,4]
Table 2. Histologic Grade of Childhood Astrocytomas and Other Tumors of Glial Origin
Type * WHO Histologic Grade
Astrocytic Tumors:
Pilocytic astrocytoma: I
Pilomyxoid astrocytoma II
Pleomorphic xanthoastrocytoma II
Subependymal giant cell astrocytoma I
Diffuse astrocytoma:
Gemistocytic astrocytoma II
Protoplasmic astrocytoma II
Fibrillary astrocytoma II
Anaplastic astrocytoma III
Glioblastoma multiforme IV
Oligodendroglial Tumors:
Oligodendroglioma II
Anaplastic oligodendroglioma III
Mixed Gliomas:
Oligoastrocytoma II
Anaplastic oligoastrocytoma III
Childhood astrocytomas and other tumors of glial origin can occur anywhere in the CNS, although each tumor type tends to have preferential CNS locations.
Table 3. Childhood Astrocytomas and Other Tumors of Glial Origin and Preferential CNS Location
Enlarge
Tumor Type * Preferential CNS location
Pilocytic astrocytoma Optic nerve, optic chiasm/hypothalamus, thalamus and basal ganglia, cerebral hemispheres, cerebellum, brain stem, spinal cord (rare)
Pleomorphic xanthoastrocytoma Superficial location in cerebrum (temporal lobe preferentially)
Diffuse astrocytoma (including fibrillary) Cerebrum (frontal and temporal lobes), brain stem, spinal cord, optic nerve, optic chiasm, optic pathway, hypothalamus, thalamus
Anaplastic astrocytoma, glioblastoma Cerebrum; occasionally cerebellum, brain stem and spinal cord
Oligodendrogliomas Cerebrum (frontal lobe preferentially followed by temporal, parietal and occipital lobes), cerebellum, brain stem, spinal cord
Oligoastrocytoma Cerebral hemispheres (frontal lobe preferentially followed by the temporal lobe)
Gliomatosis cerebri Cerebrum with or without brain stem involvement, cerebellum, spinal cord
More than 80% of astrocytomas located in the cerebellum are low-grade (pilocytic grade I) and often cystic; most of the remainder are diffuse grade II astrocytomas. Malignant astrocytomas in the cerebellum are rare.[1,2] The presence of certain histologic features has been used retrospectively to predict event-free survival for pilocytic astrocytomas arising in the cerebellum or other location.[5,6]
Children with neurofibromatosis type 1 (NF1) have an increased propensity to develop WHO grade I and II astrocytomas in the visual pathway; approximately 20% of all patients with NF1 will develop a visual pathway glioma. In these patients, the tumor may be found on screening evaluations when the child is asymptomatic or has apparent static neurologic and/or visual deficits. Pathologic confirmation is frequently not obtained in asymptomatic patients, and when biopsies have been performed, these tumors have been found to be predominantly pilocytic (grade I) rather than fibrillary (grade II) astrocytomas.[2,4,7-9] In general, treatment is not required for incidental tumors found with surveillance scans. Symptomatic lesions or those that have radiographically progressed may require treatment.[10]
Genomic alterations involving BRAF are very common in sporadic cases of pilocytic astrocytoma, resulting in activation of the ERK/MAPK pathway. BRAF activation occurs commonly through a gene fusion between KIAA1549 and BRAF producing a fusion protein that lacks the BRAF regulatory domain.[11-15] This fusion is seen in the majority of cerebellar pilocytic astrocytomas but less commonly at other sites (e.g., diencephalic, cerebral, and brain stem).[11,12,16,17] Less commonly observed genomic alterations in pilocytic astrocytomas that also activate the ERK/MAPK pathway are also seen.[12,14,15,18] Activating BRAF genomic alterations are uncommon in pilocytic astrocytoma associated with neurofibromatosis.[19]
Gliomatosis cerebri is a diffuse glioma that involves widespread involvement of the cerebral hemispheres in which it may be confined, but it often extends caudally to affect the brainstem, cerebellum and/or spinal cord.[1] It rarely arises in the cerebellum and spreads rostrally.[20] The neoplastic cells are most commonly astrocytes, but in some cases, they are oligodendroglia. Although they occur primarily in adults, more than 100 cases have been observed in children.[21] They may respond to treatment initially, but overall have a poor prognosis.
Prognosis
Low-Grade Astrocytomas
Low-grade astrocytomas (grade I [pilocytic] and grade II) have a relatively favorable prognosis, particularly if complete excision is possible.[22-26] Tumor spread, when it occurs, is usually by contiguous extension; dissemination to other central nervous system (CNS) sites is uncommon.[27] Although metastasis is uncommon, tumors may be of multifocal origin, especially when associated with neurofibromatosis type 1.
High-Grade Astrocytomas
High-grade astrocytomas are often locally invasive and extensive and tend to occur above the tentorium.[22,23,25] Spread via the subarachnoid space may occur. Metastasis outside of the CNS has been reported but is extremely infrequent until multiple local relapses have occurred. Biologic markers, such as p53 overexpression and mutation status, may be useful predictors of outcome in patients with high-grade gliomas.[4,28,29] There are data that the molecular signature of pediatric high-grade astrocytomas vary markedly from adult high-grade astrocytomas.[30] MIB-1 labeling index, a marker of cellular proliferative activity, is predictive of outcome in childhood malignant brain tumors. Both histologic classification and proliferative activity evaluation have been shown to be independently associated with survival.[31] Although high-grade astrocytoma carries a generally poor prognosis in younger patients, those with anaplastic astrocytoma and those in whom a gross total resection is possible may fare better.[26,32,33]
Disease Presentation
Presenting symptoms for childhood astrocytomas depend not only on central nervous system location, but also size of tumor, rate of growth, and chronologic and developmental age of the child.
References
Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.
Louis DN, Ohgaki H, Wiestler OD, et al.: The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114 (2): 97-109, 2007. [PUBMED Abstract]
Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [PUBMED Abstract]
Komotar RJ, Burger PC, Carson BS, et al.: Pilocytic and pilomyxoid hypothalamic/chiasmatic astrocytomas. Neurosurgery 54 (1): 72-9; discussion 79-80, 2004. [PUBMED Abstract]
Tibbetts KM, Emnett RJ, Gao F, et al.: Histopathologic predictors of pilocytic astrocytoma event-free survival. Acta Neuropathol 117 (6): 657-65, 2009. [PUBMED Abstract]
Rodriguez FJ, Scheithauer BW, Burger PC, et al.: Anaplasia in pilocytic astrocytoma predicts aggressive behavior. Am J Surg Pathol 34 (2): 147-60, 2010. [PUBMED Abstract]
Listernick R, Darling C, Greenwald M, et al.: Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. J Pediatr 127 (5): 718-22, 1995. [PUBMED Abstract]
Rosai J, Sobin LH, eds.: Atlas of Tumor Pathology. Third Series. Washington, DC : Armed Forces Institute of Pathology, 1996..
Allen JC: Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1. Pediatr Neurosurg 32 (3): 154-62, 2000. [PUBMED Abstract]
Molloy PT, Bilaniuk LT, Vaughan SN, et al.: Brainstem tumors in patients with neurofibromatosis type 1: a distinct clinical entity. Neurology 45 (10): 1897-902, 1995. [PUBMED Abstract]
Bar EE, Lin A, Tihan T, et al.: Frequent gains at chromosome 7q34 involving BRAF in pilocytic astrocytoma. J Neuropathol Exp Neurol 67 (9): 878-87, 2008. [PUBMED Abstract]
Forshew T, Tatevossian RG, Lawson AR, et al.: Activation of the ERK/MAPK pathway: a signature genetic defect in posterior fossa pilocytic astrocytomas. J Pathol 218 (2): 172-81, 2009. [PUBMED Abstract]
Jones DT, Kocialkowski S, Liu L, et al.: Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68 (21): 8673-7, 2008. [PUBMED Abstract]
Jones DT, Kocialkowski S, Liu L, et al.: Oncogenic RAF1 rearrangement and a novel BRAF mutation as alternatives to KIAA1549:BRAF fusion in activating the MAPK pathway in pilocytic astrocytoma. Oncogene 28 (20): 2119-23, 2009. [PUBMED Abstract]
Pfister S, Janzarik WG, Remke M, et al.: BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest 118 (5): 1739-49, 2008. [PUBMED Abstract]
Korshunov A, Meyer J, Capper D, et al.: Combined molecular analysis of BRAF and IDH1 distinguishes pilocytic astrocytoma from diffuse astrocytoma. Acta Neuropathol 118 (3): 401-5, 2009. [PUBMED Abstract]
Horbinski C, Hamilton RL, Nikiforov Y, et al.: Association of molecular alterations, including BRAF, with biology and outcome in pilocytic astrocytomas. Acta Neuropathol 119 (5): 641-9, 2010. [PUBMED Abstract]
Janzarik WG, Kratz CP, Loges NT, et al.: Further evidence for a somatic KRAS mutation in a pilocytic astrocytoma. Neuropediatrics 38 (2): 61-3, 2007. [PUBMED Abstract]
Yu J, Deshmukh H, Gutmann RJ, et al.: Alterations of BRAF and HIPK2 loci predominate in sporadic pilocytic astrocytoma. Neurology 73 (19): 1526-31, 2009. [PUBMED Abstract]
Rorke-Adams LB, Portnoy H: Long-term survival of an infant with gliomatosis cerebelli. J Neurosurg Pediatr 2 (5): 346-50, 2008. [PUBMED Abstract]
Armstrong GT, Phillips PC, Rorke-Adams LB, et al.: Gliomatosis cerebri: 20 years of experience at the Children's Hospital of Philadelphia. Cancer 107 (7): 1597-606, 2006. [PUBMED Abstract]
Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994. [PUBMED Abstract]
Deutsch M, ed.: Management of Childhood Brain Tumors. Boston: Kluwer Academic Publishers, 1990.
Fisher PG, Tihan T, Goldthwaite PT, et al.: Outcome analysis of childhood low-grade astrocytomas. Pediatr Blood Cancer 51 (2): 245-50, 2008. [PUBMED Abstract]
Pfister S, Witt O: Pediatric gliomas. Recent Results Cancer Res 171: 67-81, 2009. [PUBMED Abstract]
Qaddoumi I, Sultan I, Gajjar A: Outcome and prognostic features in pediatric gliomas: a review of 6212 cases from the Surveillance, Epidemiology, and End Results database. Cancer 115 (24): 5761-70, 2009. [PUBMED Abstract]
Civitello LA, Packer RJ, Rorke LB, et al.: Leptomeningeal dissemination of low-grade gliomas in childhood. Neurology 38 (4): 562-6, 1988. [PUBMED Abstract]
Pollack IF, Finkelstein SD, Woods J, et al.: Expression of p53 and prognosis in children with malignant gliomas. N Engl J Med 346 (6): 420-7, 2002. [PUBMED Abstract]
Rood BR, MacDonald TJ: Pediatric high-grade glioma: molecular genetic clues for innovative therapeutic approaches. J Neurooncol 75 (3): 267-72, 2005. [PUBMED Abstract]
Paugh BS, Qu C, Jones C, et al.: Integrated molecular genetic profiling of pediatric high-grade gliomas reveals key differences with the adult disease. J Clin Oncol 28 (18): 3061-8, 2010. [PUBMED Abstract]
Pollack IF, Hamilton RL, Burnham J, et al.: Impact of proliferation index on outcome in childhood malignant gliomas: results in a multi-institutional cohort. Neurosurgery 50 (6): 1238-44; discussion 1244-5, 2002. [PUBMED Abstract]
Finlay JL, Boyett JM, Yates AJ, et al.: Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimen. Childrens Cancer Group. J Clin Oncol 13 (1): 112-23, 1995. [PUBMED Abstract]
Villano JL, Seery TE, Bressler LR: Temozolomide in malignant gliomas: current use and future targets. Cancer Chemother Pharmacol 64 (4): 647-55, 2009. [PUBMED Abstract]
Stage Information
There is no generally recognized staging system for childhood astrocytomas. For the purposes of this summary, childhood astrocytomas will be described as low-grade astrocytoma (pilocytic astrocytomas and diffuse fibrillary astrocytomas) or high-grade astrocytoma (anaplastic astrocytomas and glioblastoma multiforme) and as untreated or recurrent.
Treatment Option Overview
Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best available, accepted therapy. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those that were previously obtained with existing therapy.
Because of the relative rarity of cancer in children, all patients with brain tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy of pediatric brain tumors is technically very demanding and should be carried out in centers that have experience in that area in order to ensure optimal results.
Debilitating effects on growth and neurologic development have frequently been observed following radiation therapy, especially in younger children.[1-3] There are also other less common complications of radiation therapy, including cerebrovascular accidents.[4] For this reason, the role of chemotherapy in allowing a delay in the administration of radiation therapy is under study, and preliminary results suggest that chemotherapy can be used to delay, and sometimes obviate, the need for radiation therapy in children with benign and malignant lesions.[5] Long-term management of these patients is complex and requires a multidisciplinary approach.
The designations that treatments are “standard” or “under clinical evaluation” are not to be used as a basis for reimbursement determinations.
References
Packer RJ, Sutton LN, Atkins TE, et al.: A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. J Neurosurg 70 (5): 707-13, 1989. [PUBMED Abstract]
Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994. [PUBMED Abstract]