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Childhood Central Nervous System Embryonal Tumors

(Also Called 'Endothelial Myeloma', 'Pineal Tumors - Pediatric', 'PNET (Primitive Neuroectodermal Tumor)', 'Primitive Neuroectodermal Tumor')

Central nervous system embryonal tumors begin in embryonic (fetal) cells in the brain and spinal cord.

Central nervous system (CNS) embryonal tumors form in brain cells when the fetus is beginning to develop. The tumors may be benign (not cancer) or malignant (cancer). Most CNS embryonal tumors in children are malignant.

Although cancer is rare in children, brain tumors are the third most common type of childhood cancer, after leukemia and lymphoma. This summary discusses the treatment of primary brain tumors (tumors that begin in the brain). The treatment of metastatic brain tumors, which 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.

CNS embryonal tumors may form in different areas of the brain.

Childhood brain tumors are named based on the type of cell they formed in and where the tumor first formed in the brain. There are 6 different types of CNS embryonal tumors:

Medulloblastoma

Medulloblastomas are fast-growing tumors that form in brain cells in the cerebellum. The cerebellum is at the lower back part of the brain between the cerebrum and the brain stem. The cerebellum controls movement, balance, and posture. Sometimes medulloblastoma spreads to the bone, bone marrow, lymph nodes, liver, or lung.

Pineoblastoma

Pineoblastoma is a fast-growing tumor that forms in brain cells in or near the pineal gland. The pineal gland is a tiny organ in the brain that makes melatonin, a substance that helps control our sleep cycle.

Pineal parenchymal tumors

Pineal parenchymal tumors form in pineocytes, a type of cell in the pineal gland. These tumors may be slow or fast-growing.

Supratentorial primitive neuroectodermal tumors

Supratentorial primitive neuroectodermal tumors are fast-growing tumors that form in brain cells in the cerebrum. The cerebrum is at the top of the head and is the largest part of the brain. The cerebrum controls thinking, learning, problem-solving, emotions, speech, reading, writing, and voluntary movement.

Medulloepithelioma

Medulloepitheliomas are fast-growing tumors that form in brain cells that line tubelike spaces in the brain and spinal cord. These rare tumors are most common in infants and young children.

Ependymoblastoma

Ependymoblastomas are fast-growing tumors that form in brain cells lining the fluid -filled spaces in the brain and spinal cord. These rare tumors are most common in infants and young children.

Childhood CNS atypical teratoid/rhabdoid tumor is also a type of embryonal tumor, but it is treated differently than other childhood CNS embryonal tumors.

Certain genetic conditions increase the risk of childhood CNS embryonal tumors.

Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Parents who think their child may be at risk should discuss this with the child's doctor. Risk factors for CNS embryonal tumors include having the following inherited diseases:

  • Li-Fraumeni syndrome.
  • Nevoid basal cell carcinoma (Gorlin) syndrome.
  • Turcot syndrome.

In most cases, the cause of CNS embryonal tumors is not known.

The symptoms of childhood CNS embryonal tumors are not the same in every child.

The following symptoms and others may be caused by a CNS embryonal tumor. Symptoms vary depending on the child's age and where the tumor is located. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Loss of balance, trouble walking, worsening handwriting, or slow speech.
  • Morning headache or headache that goes away after vomiting.
  • Nausea and vomiting.
  • General weakness or weakness on one side of the face.
  • Unusual sleepiness or change in energy level.
  • Change in personality or behavior.
  • Unexplained weight loss or weight gain.

An infant or young child may be irritable or grow slowly, and may not eat well or meet developmental milestones such as sitting, walking, and talking in sentences.

Tests that examine the brain and spinal cord are used to detect (find) childhood CNS embryonal tumors.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a patient's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • 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) of the brain and spinal cord 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). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue.
  • Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
  • SPECT scan (single photon emission computed tomography): A procedure that uses a special camera linked to a computer to make a 3-dimensional (3-D) picture of the brain. A small amount of a radioactive substance is injected into a vein or inhaled through the nose. As the substance travels through the blood, the camera rotates around the head and takes pictures of the brain. There will be increased blood flow and more chemical reactions (metabolism) in areas where cancer cells are growing. These areas will show up brighter in the picture. This procedure may be done just before or after a CT scan.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the brain. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

Childhood CNS embryonal tumors are usually diagnosed and removed in surgery.

If doctors think your child may have a CNS embryonal tumor, a biopsy may be done to remove a sample of tissue. For brain tumors, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. Sometimes, a computer-guided needle is used to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery.

The following tests may be done on the sample of tissue that is removed:

  • Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
  • Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
  • Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

Some pineoblastoma and pineal parenchymal tumors are diagnosed by brain imaging tests.

Sometimes, it is not possible to do a safe biopsy or completely remove the tumor in surgery, because of where it is in the brain. These tumors are diagnosed based on the results of imaging tests and other procedures.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on:

  • The age of the child when the tumor is found.
  • The type of tumor and where it is in the brain.
  • Whether there is tumor left after surgery.
  • Whether the cancer has spread within the brain and spinal cord or to other parts of the body, such as the bones.
  • Whether the tumor has just been diagnosed or has recurred (come back).

Staging Childhood Central Nervous System Embryonal Tumors

Treatment is based on the child’s risk group.

Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment.

There is no standard staging system for childhood central nervous system (CNS) embryonal tumors. Instead, treatment depends on the child’s risk group:

Average risk

Childhood CNS embryonal tumors are called average risk when all of the following are true:

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

High risk

Childhood CNS embryonal tumors are called high risk if any of the following are true:

  • The child is 3 years of age or younger.
  • The tumor is not at the very back of the brain.
  • Some of the tumor was not removed by surgery.
  • The cancer has spread to other parts of the body.

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

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The information from tests and procedures done to detect (find) childhood CNS embryonal tumors is used to plan cancer treatment.

Some of the tests used to detect childhood CNS embryonal tumors are repeated after the tumor is removed by surgery. This is to find out how much tumor remains after surgery. Other tests and procedures may be done to find out if the cancer has spread:

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
  • Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
  • 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.

Recurrent Childhood Central Nervous System Embryonal Tumors

A recurrent childhood central nervous system (CNS) embryonal tumor is a tumor that recurs (comes back) after being treated. Childhood CNS embryonal tumors most often recur within 18 months after treatment but may come back many years later. Recurrent childhood CNS embryonal tumors may come back in the same place as the original tumor or in a different place in the brain or spinal cord.

Treatment Option Overview

There are different types of treatment for children who have CNS embryonal tumors.

Different types of treatment are available for children with central nervous system (CNS) embryonal tumors. 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. Some clinical trials are open only to patients who have not started treatment.

Children who have CNS embryonal tumors should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts 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
  • Psychologist

Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years.

Symptoms caused by the tumor may begin before diagnosis. These symptoms may continue for months or years. It is important to talk with your child's doctors about symptoms caused by the tumor that may continue after treatment.

Some cancer treatments cause side effects months or years after treatment has ended.

Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

Children diagnosed with medulloblastoma may have cerebellar mutism syndrome after surgery. Symptoms of this syndrome include the following:

  • Not being able to speak.
  • Trouble swallowing and eating.
  • Loss of balance, trouble walking, and worsening handwriting.
  • Loss of muscle tone.
  • Mood swings and changes in personality.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child.

Three types of standard treatment are used:

Surgery

Surgery is used to diagnose and treat a childhood CNS embryonal tumor as described in this summary.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery to lower the risk that the cancer will come back is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. 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.

Radiation therapy to the brain can affect growth and development in young children. For this reason, clinical trials are studying new ways of giving radiation that may have fewer side effects than standard methods. For childhood CNS embryonal tumors, radiation therapy may be given in the following ways:

  • Conformal radiation therapy uses a computer to make a 3-dimensional (3-D) picture of the tumor and the radiation beams are shaped to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to normal tissue around the tumor.
  • Stereotactic radiation therapy uses a rigid head frame attached to the skull to aim radiation directly to a tumor, causing less damage to normal tissue around the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.

The way the radiation therapy is given depends on the type of cancer being treated.

Because radiation therapy can affect growth and brain development in young children, especially children who are three years old or younger, chemotherapy may be given to delay or reduce the need for radiation therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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 into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated.

Anticancer drugs given by mouth or vein to treat central nervous system tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells that may have spread there. This is called intrathecal chemotherapy.

New types of treatment are being tested in clinical trials.

This section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the brain tumor has recurred (come back). If the tumor recurs in the brain, a biopsy may also be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Childhood Central Nervous System Embryonal Tumors

For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Newly Diagnosed Childhood Medulloblastoma

In newly diagnosed childhood medulloblastoma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Average risk

Standard treatment of average-risk childhood medulloblastoma is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Clinical trials are studying new combinations of radiation therapy and chemotherapy.

High risk

Standard treatment of high-risk childhood medulloblastoma is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Clinical trials are studying new combinations of radiation therapy and chemotherapy.

Children 3 years old or younger

Standard treatment of childhood medulloblastoma in children 3 years old or younger is usually surgery followed by chemotherapy. Other treatments may include the following:

  • Surgery followed by combination chemotherapy.
  • Surgery followed by high-dose chemotherapy with stem cell transplant.
  • Surgery followed by combination chemotherapy and radiation therapy to the area where the tumor was removed.

Treatment of medulloblastoma in children 3 years old or younger is often within a clinical trial. Clinical trials are studying new combinations of chemotherapy with stem cell transplant.

Newly Diagnosed Childhood Pineoblastoma and Pineal Parenchymal Tumors

In newly diagnosed childhood pineoblastoma or pineal parenchymal tumors, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Children older than 3 years

Standard treatment of childhood pineoblastoma and pineal tumors in children older than 3 years is usually surgery. It is usually not possible to remove all of the tumor because of where it is in the brain. Surgery is usually followed by radiation therapy to the brain and spinal cord.

Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Clinical trials are studying new treatments for high-risk childhood pineoblastoma, including combinations of chemotherapy, radiation therapy, and stem cell transplant.

Children 3 years old or younger

Treatment of pineoblastoma and pineal tumors in children 3 years old or younger may include surgery followed by chemotherapy. Radiation therapy may be given when the child is older. High-dose chemotherapy with stem cell transplant has been used.

Newly Diagnosed Childhood Supratentorial Primitive Neuroectodermal Tumors

In newly diagnosed childhood supratentorial primitive neuroectodermal tumors, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Children older than 3 years

Standard treatment of supratentorial neuroectodermal tumors in children older than 3 years is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Children 3 years old or younger

Standard treatment of supratentorial neuroectodermal tumors in children 3 years old or younger is usually surgery followed by chemotherapy. Other treatments may include the following:

  • Surgery followed by combination chemotherapy.
  • Surgery followed by high-dose chemotherapy with stem cell transplant.
  • Surgery followed by combination chemotherapy and radiation therapy to the area where the tumor was removed.

Treatment of supratentorial primitive neuroectodermal tumors in children 3 years old or younger is often within a clinical trial. Clinical trials are studying new combinations of chemotherapy with stem cell transplant.

Newly Diagnosed Childhood Medulloepithelioma and Ependymoblastoma

In newly diagnosed childhood medulloepithelioma and ependymoblastoma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Children older than 3 years

Standard treatment of childhood medulloepithelioma or ependymoblastoma in children older than 3 years is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Children 3 years old or younger

Standard treatment of childhood medulloepithelioma or ependymoblastoma in children 3 years old or younger is usually surgery followed by chemotherapy. Other treatments may include the following:

  • Surgery followed by combination chemotherapy.
  • Surgery followed by high-dose chemotherapy with stem cell transplant.
  • Surgery followed by combination chemotherapy and radiation therapy to the area where the tumor was removed.

Treatment of childhood medulloepithelioma or ependymoblastoma in children 3 years old or younger is often within a clinical trial.

Recurrent Childhood Central Nervous System Embryonal Tumors

Treatment of recurrent childhood CNS embryonal tumors may include the following:

  • For patients who previously received radiation therapy and chemotherapy, treatment may be chemotherapy and/or stereotactic radiation therapy.
  • For infants and young children who previously received chemotherapy only and have a local recurrence, treatment may be chemotherapy with radiation therapy to the tumor and the area close to it.
  • For patients who previously received radiation therapy, high-dose chemotherapy and stem cell transplant may be used.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with childhood central nervous system embryonal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

For more information from the NCI, please write to this address:

NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322

U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1.800.4-CANCER (1.800.422.6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1.800.332.8615. Information about ongoing clinical trials is available from the NCI Web site.

Source: National Institutes of Health; National Cancer Institute

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 8/12/2009...#6136


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