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.

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.

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:

  • 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): 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 the patient through 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 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:

  • Size and spread of the tumor before surgery.
  • Cancer cells remaining after surgery.

Treatment options depend on:

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

Stages of Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

After the childhood supratentorial primitive neuroectodermal tumor or pineoblastoma has been removed, tests are done to find out if there is tumor remaining.

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:

  • MRI (magnetic resonance imaging): 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 the patient through 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).
  • 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.

The following risk categories are used for childhood supratentorial primitive neuroectodermal tumors and pineoblastoma:

Average risk

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

  • The child is older than 3 years.
  • All of the tumor was removed by surgery or there was a very small amount remaining.
  • The cancer has not spread to other parts of the brain or body, including the spinal cord.

Poor risk

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

  • The child is younger than 3 years.
  • The tumor is near the center of the brain.
  • Some of the tumor was not removed by surgery.
  • The cancer has spread to other parts of the brain or body, including the spinal cord.

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

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 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.

Children with supratentorial primitive neuroectodermal tumors and pineoblastoma should have their treatment planned by a team of doctors with expertise in treating childhood brain tumors.

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:

  • Neurosurgeon.
  • Neurologist.
  • Neuropathologist.
  • Neuroradiologist.
  • Rehabilitation specialist.
  • Radiation oncologist.
  • Medical oncologist.
  • Endocrinologist.
  • Psychologist.

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. 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 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.

Treatment Options for Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

Untreated Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

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:

  • 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.

Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma

 

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.

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

  • Surgery.
  • Chemotherapy.

Source:  National Cancer Institute