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

Childhood supratentorial primitive 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 cerebral neuroblastomas 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. (Refer to the PDQ treatment summary on Adult Brain Tumors for more information.)

These symptoms may be caused by a supratentorial primitive neuroectodermal tumor, a pineoblastoma, or by other conditions. A doctor should be consulted if any of the following problems occur:

The following tests and procedures may be used:

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.

The prognosis (chance of recovery) depends on:

Treatment options depend on:

The extent or spread of cancer is usually described as stages. For childhood supratentorial primitive neuroectodermal tumors and pineoblastoma, risk groups are used instead of stages. Risk groups are described by the amount of remaining tumor or spread of cancer cells within the central nervous system (brain and spinal cord) or to other parts of the body. It is important to know the risk group in order to plan treatment. The following tests and procedures may be used in determining the risk group:

Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are called average risk if all of the following are true:

Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are called poor risk if any of the following are true:

In general, cancer is more likely to recur (come back) in patients in the poor risk group.

Recurrent childhood supratentorial primitive 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.

Different types of treatment are available for children with supratentorial primitive 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. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Cancer.gov Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Your child’s treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist may refer you to other pediatric doctors who have experience and expertise in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

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 is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. 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 is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells 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, a body cavity such as the abdomen, or an organ, the drugs mainly affect cancer cells in those areas. 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.

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

Untreated childhood supratentorial primitive 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:

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:

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

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.

Information about these and other ongoing clinical trials is available from the NCI Cancer.gov Web site.

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

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

New treatments are being studied in clinical trials for recurrent childhood supratentorial primitive neuroectodermal tumors and pineoblastoma. Information about this and other ongoing clinical trials is available from the NCI Cancer.gov Web site.

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

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

Editorial changes were made to this summary.

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.

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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.