Key Points for This Section

Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are tumors in which malignant (cancer) cells form in the tissues of the brain. 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. Tests that examine the brain and spinal cord are used to detect (find) childhood supratentorial primitive neuroectodermal tumors and pineoblastoma. Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are usually diagnosed and removed in surgery. Certain factors affect prognosis (chance of recovery) and treatment options.

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 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. See the following PDQ treatment summaries for more information:

• Childhood Brain and Spinal Cord Tumors Treatment Overview ¹
• Adult Brain Tumors ²

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).

Glossary Terms

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.

The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (non-cancerous) or malignant (cancerous).

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is 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.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

One half of the cerebrum, the part of the brain that controls muscle functions and also controls speech, thought, emotions, reading, writing, and learning. The right hemisphere controls the muscles on the left side of the body, and the left hemisphere controls the muscles on the right side of the body.

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.

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.

Computed tomography 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 computed tomography scan, computerized tomography, computerized axial tomography scan, and CAT scan.

The process of identifying a disease by the signs and symptoms.

A substance that is being studied in the treatment of cancer. It may make tumor cells more sensitive to radiation therapy, improve tumor images using magnetic resonance imaging (MRI), and kill cancer cells. It is a type of metalloporphyrin complex. Also called motexafin gadolinium.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

A malignant brain tumor that begins in the lower part of the brain and that can spread to the spine or to other parts of the body. Medulloblastomas are a type of primitive neuroectodermal tumor (PNET).

A hormone made by the pineal gland (tiny organ near the center of the brain). Melatonin helps control the body’s sleep cycle, and is an antioxidant. It is also made in the laboratory and sold as a supplement.

A very thin layer of tissue that covers a surface.

The three thin layers of tissue that cover and protect the brain and spinal cord.

Cancer that has spread from the place in which it started to other parts of the body.

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, nuclear magnetic resonance imaging, and NMRI.

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.

Cancer that arises in immature nerve cells and affects mostly infants and children.

A part of the body that performs a specific function. For example, the heart is an organ.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

A tiny organ in the cerebrum that produces melatonin. Also called pineal body or pineal organ.

A fast growing type of brain tumor that occurs in or around the pineal gland, a tiny organ near the center of the brain.

The original tumor.

One of a group of cancers that develop from the same type of early cells, and share certain biochemical and genetic features. Some primitive neuroectodermal tumors develop in the brain and central nervous system (CNS-PNET), and others develop in sites outside of the brain such as the limbs, pelvis, and chest wall (peripheral PNET). Also called PNET.

The likely outcome or course of a disease; the chance of recovery or recurrence.

To come back or to return.

Convulsion; a sudden, involuntary movement of the muscles.

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.

Having to do with the upper part of the brain.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A group or layer of cells that work together to perform a specific function.

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 cancerous), or malignant (cancerous). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

To eject some or all of the contents of the stomach through the mouth.

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.