Table of Contents General Information About Atypical Teratoid/Rhabdoid Tumor Stages of Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment Option Overview Treatment Options for Newly Diagnosed Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for Recurrent Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor To Learn More about Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor and Other Childhood Brain Tumors Get More Information From NCI Changes to This Summary (01/05/2009) About PDQ
General Information About Atypical Teratoid/Rhabdoid Tumor
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Central nervous system atypical teratoid/rhabdoid tumor is a disease in which malignant (cancer) cells form in the tissues of the brain.
Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children younger than three years of age, although it can occur in older children and adults.
About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that controls movement, balance, and posture. The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. AT/RT may also be found in other parts of the central nervous system (brain and spinal cord).
This summary describes the treatment of primary brain tumors (tumors that begin in the
brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that
begin in other parts of the body and spread to the brain, is not discussed in
this summary. For more information see the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview 1 about the different types of childhood brain and spinal cord tumors.
Brain tumors can occur in both children and adults; however,
treatment for children may be different than treatment for adults. See
the PDQ treatment summary on Adult Brain Tumors 2 for more information.
Certain genetic changes may increase the risk of atypical teratoid/rhabdoid tumor.
Anything that increases the 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 for cancer should discuss this with their child's doctor.
Atypical teratoid/rhabdoid tumor may be linked to a change in a tumor suppressor gene called INI1. This type of gene makes a protein that helps control cell growth. Changes in the DNA of tumor suppressor genes like INI1 may lead to cancer.
Changes in the INI1 gene may be inherited (passed on from parents to offspring). When the INI1 gene change is inherited, tumors may form in two parts of the body at the same time (for example, in the brain and the kidney). If AT/RT is diagnosed, genetic counseling (a discussion with a trained professional about inherited diseases and a possible need for gene testing) may be recommended.
The symptoms of atypical teratoid/rhabdoid tumor are not the same in every patient.
Because atypical teratoid/rhabdoid tumor is fast growing, symptoms may develop quickly and progress over a period of days or weeks. Symptoms vary and depend on the age of the patient and where the tumor has formed.
These symptoms may be caused by AT/RT or by other conditions. A doctor should be consulted if any of the following problems occur:
- Morning headache or headache that goes away after vomiting.
- Nausea and vomiting.
- Unusual sleepiness or change in activity level.
- Loss of balance, lack of coordination, or trouble walking.
- Increase in head size (in infants).
Tests that examine the brain and spinal cord are used to detect (find) atypical teratoid/rhabdoid tumor.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including 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.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen or pelvis, 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.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. A renal ultrasound is used to check for AT/RT that may develop in the kidneys at the same time as in the brain.
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INI1 gene testing: A laboratory test in which a sample of blood or tissue is tested for the INI1 gene.
Childhood atypical teratoid/rhabdoid tumor is diagnosed and may be removed in surgery.
If doctors think there might be a brain tumor, 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 a sample of tissue. 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. The pathologist checks the cancer cells to find out the type of brain tumor. It is often difficult to completely remove AT/RT because of where the tumor is in the brain and because it may already have spread at the time of diagnosis.
In order to tell the difference between AT/RT and other brain tumors, an immunohistochemistry study may be done on the sample of tissue that is removed. An immunohistochemistry study is 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.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- The age of the child.
- The amount of tumor remaining after surgery.
- Whether the cancer has spread to other parts of the central nervous system (brain and spinal cord) or to the kidney.
Stages of Central Nervous System Atypical Teratoid/Rhabdoid Tumor
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There is no standard staging system for central nervous system atypical teratoid/rhabdoid tumor.
The extent or spread of cancer is usually described as stages. There is no standard staging system for central nervous system atypical teratoid/rhabdoid tumor. This tumor is classified as newly diagnosed or recurrent. Treatment depends on how much cancer remains after surgery and the age of the child. Results from the following procedures are used to plan treatment:
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
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.
Treatment Option Overview
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There are different types of treatment for patients with central nervous system atypical teratoid/rhabdoid tumor.
Different types of treatment are available for patients with central nervous system atypical teratoid/rhabdoid tumor. Treatment for AT/RT is usually within a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer.
Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site 3. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
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 clinical trials database.
Children with atypical teratoid/rhabdoid tumor should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with central nervous system cancer and who specialize in certain areas of medicine. These may include the following specialists:
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. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer 4 for more information).
Four types of treatment are used:
Surgery
Surgery is used to diagnose and treat atypical teratoid/rhabdoid tumor. See the General Information 5 section of this summary.
Even if the doctor removes all the cancer that can be seen at the time of the surgery, most patients will be given chemotherapy and possibly radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to increase the chances of a cure, is called adjuvant 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 spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).
The way the chemotherapy is given depends on the type of cancer being treated.
Anticancer drugs given by mouth or vein to treat brain and spinal cord tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells there. This is called intrathecal chemotherapy.
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 of tumor being treated and whether it has spread. External radiation therapy may be given to the brain and spinal cord.
Because radiation therapy can affect growth and brain development in young children, especially children who are three years old or younger, the dose of radiation therapy may be lower than usual.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a method 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.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer may be repeated. 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.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site 3.
Treatment Options for Newly Diagnosed Central Nervous System Atypical Teratoid/Rhabdoid Tumor
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There is no standard treatment for patients with central nervous system atypical teratoid/rhabdoid tumor.
Combinations of treatments are used for patients with atypical teratoid/rhabdoid tumor.
Because atypical teratoid/rhabdoid tumor is fast-growing, a combination of treatments is usually given. Most treatments include both surgery and chemotherapy. Treatments for AT/RT may include combinations of the following:
Clinical trials of new treatments should be considered for patients with newly diagnosed atypical teratoid/rhabdoid tumor.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood atypical teratoid/rhabdoid tumor 6. 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 7. Treatment for Recurrent Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor
There is no standard treatment for patients with recurrent childhood central nervous system atypical teratoid/rhabdoid tumor.
Clinical trials of new treatments should be considered for patients with recurrent atypical teratoid/rhabdoid tumor.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood atypical teratoid/rhabdoid tumor 6. 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 7. To Learn More about Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor and Other Childhood Brain Tumors
For more information from the National Cancer Institute about childhood central nervous system atypical teratoid/rhabdoid tumor and other childhood brain tumors, see the following:
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
Get More Information From NCI
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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.
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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 23. 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 (01/05/2009)
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.
This is a new 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 22. 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 24. 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 22 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. |