Supratentorial Primitive Neuroectodermal Tumors (Childhood)


General Information

What are childhood primitive neuroectodermal tumor and pineal tumor?

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

Brain tumors are grouped by their location within the brain and the type of brain cells where the cancer began. Supratentorial tumors are found in the upper part of the brain. Childhood supratentorial primitive neuroectodermal tumors are supratentorial tumors. Pineal region tumors are tumors found in or around a tiny organ located near the center of the brain (the pineal gland).

This treatment information summary covers tumors that start in the brain (primary brain tumors). Often cancer found in the brain has started somewhere else in the body and has spread (metastasized) to the brain. This is called brain metastases. (Refer to Adult Brain Tumor Treatment for more information.)

A magnetic resonance imaging (MRI) scan, which uses magnetic waves to make a picture of your child's brain, may be done.

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

There are many types of brain tumors in children and the chance of recovery (prognosis) depends on the type of tumor, where it is located within the brain, and your child's age and general health.

Stage Information

Once childhood supratentorial primitive neuroectodermal tumor or pineal tumor is found, more tests will be done to find out the type of tumor. If a biopsy specimen is taken, the cancer cells will be looked at carefully under a microscope to see how different they are from the normal cells. This will determine the histologic grade of the tumor. Your child's doctor needs to know the type and grade of tumor in order to plan treatment.

Untreated childhood pineal tumor
The tumors can be slow growing (pineocytomas) or fast growing (pineoblastomas). They can spread to other parts of the brain and spinal cord. Pineoblastoma can also spread to organs outside of the brain.

Untreated childhood supratentorial primitive neuroectodermal tumor
Supratentorial primitive neuroectodermal tumors occur in the upper part of the brain. The cancer cells look very little like other brain cells. This kind of cancer is sometimes called a cerebral neuroblastoma. It can spread to other parts of the brain and spinal cord.

Recurrent childhood pineal tumor
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the brain orin another part of the body.

Recurrent childhood supratentorial primitive neuroectodermal tumor
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the brain or in another part of the body.

Treatment Option Overview

There are treatments for all children with childhood supratentorial neuroectodermal tumors and pineal tumors.

Three kinds of treatment are used:

-surgery (taking out the cancer in an operation)
-radiation therapy (using high-dose x-rays to kill cancer cells)
-chemotherapy (using drugs to kill cancer cells).

Surgery is the most common treatment for a child with supratentorial neuroectodermal tumors or pineal tumors. Depending on where the cancer is and the type of cancer, your child's doctor may remove as much of the tumor as possible. If the tumor cannot be totally removed, radiation therapy and chemotherapy may also be given. If the cancer is in a place where it cannot be removed, surgery may be limited to a biopsy of the cancer.

Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation therapy for childhood supratentorial neuroectodermal tumors and pineal tumors usually comes from a machine outside the body (external radiation therapy). The use of radiation put into the brain through thin plastic tubes (internal radiation therapy) is under study. For some types of brain tumors, clinical trials are evaluating radiation therapy given in several small doses each day (hyperfractionated radiation therapy). Radiation therapy can affect growth and brain development, so clinical trials are testing ways to decrease or delay radiation therapy, especially for younger children.

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

Treatment By Type

Treatment for childhood supratentorial neuroectodermal tumors and pineal tumors depends on the type and stage of the disease and your child's age and overall health.

Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child go into a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients. Clinical trials are ongoing in most parts of the country for childhood supratentorial neuroectodermal tumors and pineal tumors.

Untreated Childhood SPNET and Pineal Tumors
SPNET: Supratentorial Primitive Neuroectodermal Tumor

Your child's treatment will probably be surgery to remove as much of the cancer as possible. After surgery, your child's treatment depends on the risk of the cancer coming back and the child's age.

If your child is older than 3 years of age, the cancer is in the very back part of the brain, there is no or only a very small amount of cancer left after surgery, and no sign that the cancer has spread, the risk is called "average" that the cancer will come back (recur). After surgery, your child will probably receive radiation therapy.

If your child is younger than 3 years of age, the cancer is not in the very back part of the brain, all of the cancer could not be removed during surgery, or there is spread to other parts of the brain, the risk is higher that the cancer will recur. After surgery, your child will probably receive radiation therapy and chemotherapy. Clinical trials are evaluating whether chemotherapy can be given to children under the age of 3 to delay or reduce radiation therapy. Clinical trials are evaluating new treatment options for these patients.

Recurrent Childhood SPNET and Pineal Tumors
SPNET: Supratentorial Primitive Neuroectodermal Tumor

Treatment for recurrent disease depends on the type of tumor, whether the tumor comes back in the same place or in another part of the brain, and the treatment that was given before. You may want to consider entering your child on a clinical trial of new treatments.






The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals.

The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation.

The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. Any use of the information will be at the risk of the user.





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