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
• 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).

Back to Top

Next Section >