Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment (PDQ®)

General Information About Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

Note: This PDQ summary contains content that is included in the new PDQ Childhood Central Nervous System Embryonal Tumors summary. A health professional version of the Childhood Central Nervous System Embryonal Tumors summary is currently available on the National Cancer Institute (NCI) Web site and a patient version is coming soon.

In the near future, the health professional and patient versions of the PDQ Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma summary will be removed from the NCI Web site.

Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are tumors in which malignant (cancer) cells form in the tissues of the brain.

Childhood supratentorialprimitive neuroectodermal tumors start in the cerebrum. The cerebrum, which is at the top of the head, is the largest part of the brain. The cerebrum controls thinking, learning, problem solving, speech, emotions, reading, writing, and voluntary movement. Childhood supratentorial primitive neuroectodermal tumors are also called cerebralneuroblastomas or cerebral medulloblastomas.

Pineoblastoma form in or near the pineal gland. The pineal gland is a tiny organ in the brain that produces melatonin, a substance that helps control our sleeping and waking cycle.

Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia and lymphoma.

This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment of 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.

Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the following PDQ treatment summaries for more information:

• Childhood Brain and Spinal Cord Tumors Treatment Overview

• Adult Brain Tumors

The cause of most childhood brain tumors is unknown.

The symptoms of childhood supratentorial primitive neuroectodermal tumors and pineoblastoma vary and often depend on the child’s age, where the tumor is located, and the size of the tumor.

The following symptoms and others may be caused by a supratentorial primitive neuroectodermal tumor or a pineoblastoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur:

• Weakness or change in sensation on one side of the body.

• Morning headache or headache that goes away after vomiting.

• Nausea and vomiting.

• Seizures.

• Unusual sleepiness or change in energy level.

• Change in personality or behavior.

• Unexplained weight loss or weight gain.

Tests that examine the brain and spinal cord are used to detect (find) childhood supratentorial primitive neuroectodermal tumors and pineoblastoma.

The following tests and procedures may be used:

• 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.

• 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 areas inside 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).

Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are usually diagnosed and removed in surgery.

If a brain tumor is suspected, a biopsy is done by removing part of the skull and using a needle to remove a sample of brain tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) depends on:

• The size and spread of the tumor before surgery.

• Whether cancer cells remain after surgery.

Treatment options depend on:

• The age of the child when the tumor is found.

• Where the tumor is in the brain.

• The amount of tumor remaining after surgery.

• Whether the cancer has spread to other parts of the central nervous system, such as the meninges (membranes covering the brain) and spinal cord.

• Whether the cancer has spread to other parts of the body, such as the bone or lung.

• Whether the tumor has just been diagnosed or has recurred (come back).

Stages of Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

The area where the tumor is found and the child’s age are 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 supratentorialprimitive neuroectodermal tumors and pineoblastomas. Instead, the plan for cancer treatment depends on:

• Whether the tumor first formed in the cerebrum or in the pineal body.

• Whether the child was younger than three years old when the tumor was diagnosed.

The information from tests and procedures done to detect (find) childhood supratentorial primitive neuroectodermal tumors and pineoblastomas is used to plan cancer treatment.

Some of the tests used to detect childhood supratentorial primitive neuroectodermal tumors and pineoblastomas are repeated after the tumor is removed by surgery. (See the General Information section.) This is to find out how much tumor remains after surgery. Other tests and procedures may be done to find out if the cancer has spread:

• Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. A pathologist views the fluid under a microscope to look for cancer cells. This procedure is also called an LP or spinal tap.

• Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.

• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

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 Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

Recurrent childhood supratentorialprimitive neuroectodermal tumors and pineoblastoma are tumors that have recurred (come back) after they have been treated. Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma often recur. A tumor may come back many years later, usually in the brain, meninges (membranes covering the brain), or spinal cord. It can also come back in other parts of the body, such as the bone or lung.

Treatment Option Overview

There are different types of treatment for children with supratentorial primitive neuroectodermal tumors and pineoblastoma.

Different types of treatment are available for children with supratentorialprimitive neuroectodermal tumors and pineoblastoma. 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 supratentorial primitive neuroectodermal tumors and pineoblastoma 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 pediatriconcologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

• Neurosurgeon.

• Neurologist.

• Neuropathologist.

• Neuroradiologist.

• Rehabilitation specialist.

• Radiation oncologist.

• Medical oncologist.

• Endocrinologist.

• Psychologist.

Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.

Childhood brain and spinal cordtumors may cause symptoms that continue for months or years. Symptoms caused by the tumor may begin before diagnosis. Symptoms caused by treatment may begin during or right after treatment.

Some cancer treatments cause side effects months or years after treatment has ended.

These are called late effects. Late effects 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 for more information).

Three types of standard treatment are used:

Surgery

Surgery is used to diagnose and treat childhood supratentorial primitive neuroectodermal tumors and pineoblastoma as described in the General Information section of this summary.

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 stage of the cancer being treated.

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 in 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 and stage of the cancer being treated.

Because radiation therapy to the brain can affect growth and brain development in young children, clinical trials are studying ways of using chemotherapy to delay or reduce the need for radiation therapy.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

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.

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. 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 is sometimes called re-staging.

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 condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

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 Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

Untreated childhood supratentorialprimitive neuroectodermal tumors and pineoblastoma are tumors for which no treatment has been given. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Standard treatment of supratentorial neuroectodermal tumors or pineoblastoma in children 3 years of age and older may include the following:

• Surgery.

• Surgery followed by radiation therapy to the whole brain and spinal cord.

• Surgery followed by radiation therapy to the whole brain and spinal cord, and chemotherapy.

Some of the treatments being studied in clinical trials for supratentorial neuroectodermal tumors or pineoblastoma in children 3 years of age and older include the following:

• A clinical trial of chemotherapy given before or after radiation therapy.

• A clinical trial of chemotherapy to delay or reduce the need for radiation therapy.

Standard treatment of supratentorial neuroectodermal tumors or pineoblastoma in children younger than 3 years of age may include the following:

• Surgery.

• Surgery followed by chemotherapy.

One of the treatments being studied in clinical trials for supratentorial neuroectodermal tumors or pineoblastoma in children younger than 3 years of age includes chemotherapy to delay or reduce the need for radiation therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with untreated childhood supratentorial primitive neuroectodermal tumor and untreated childhood pineoblastoma.

Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

Standard treatment of recurrent childhood supratentorialprimitive neuroectodermal tumors and pineoblastoma may include the following:

• Surgery.

• Chemotherapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood supratentorial primitive neuroectodermal tumor and recurrent childhood pineoblastoma.

To Learn More About Childhood Brain Tumors

For more information from the National Cancer Institute about childhood brain tumors, see the following:

• What You Need to Know About™ Brain Tumors

• Pediatric Brain Tumor Consortium (PBTC)

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview

• Childhood Cancers

• CureSearch - National Childhood Cancer Foundation Children’s Oncology Group

• Late Effects of Treatment for Childhood Cancer

• Young People with Cancer: A Handbook for Parents

• Care for Children and Adolescents with Cancer: Questions and Answers

• Understanding Cancer Series: Cancer

• Staging: Questions and Answers

• Coping with Cancer

• Support and Resources

• Cancer Library

• Information for Survivors/Caregivers/Advocates

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® 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 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. 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 (07/03/2008)

The PDQcancer 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.

Changes were made to this summary to match those made to the health professional version.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

• Text describing the way cancer spreads in the body was added to the Stages section.

• Information about patients taking part in clinical trials and about follow-up tests was added to the Treatment Option Overview section.

• Links to ongoing clinical trials listed in NCI's PDQ Cancer Clinical Trials Registry were added to the Treatment Options section.

• A new section called "To Learn More" was added. It includes links to more information about this type of cancer and about cancer in general.

• The "Get More Information from NCI" section (originally called "To Learn More") was revised.

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. 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. 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 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.