Key Points for This Section

A childhood brain or spinal cord tumor is a disease in which abnormal cells form in the tissues of the brain or spinal cord. The brain controls many important body functions. The spinal cord connects the brain with nerves in most parts of the body. Brain and spinal cord tumors are a common type of childhood cancer. There are different types of childhood brain and spinal cord tumors. Astrocytomas Atypical Teratoid/Rhabdoid Tumor Brain Stem Glioma Central Nervous System Embryonal Tumor Central Nervous System Germ Cell Tumor Craniopharyngioma Ependymoma Medulloblastoma Spinal Cord Tumors Supratentorial Primitive Neuroectodermal Tumor Visual Pathway and Hypothalamic Glioma The cause of most childhood brain and spinal cord tumors is unknown. The symptoms of childhood brain and spinal cord tumors are not the same in every child. Tests that examine the brain and spinal cord are used to detect (find) childhood brain and spinal cord tumors. Most childhood brain tumors are diagnosed and removed in surgery. Some childhood brain and spinal cord tumors are diagnosed by imaging tests. Certain factors affect prognosis (chance of recovery).

A childhood brain or spinal cord tumor is a disease in which abnormal cells form in the tissues of the brain or spinal cord.

There are many types of childhood brain and spinal cord tumors. The tumors are formed by the abnormal growth of cells and may begin in different areas of the brain or spinal cord. Tumors may be benign (noncancerous) or malignant (cancerous).

Together, the brain and spinal cord make up the central nervous system (CNS).

The brain controls many important body functions.

The brain has three major parts:

• The cerebrum is the largest part of the brain. It is at the top of the head. The cerebrum controls thinking, learning, problem solving, emotions, speech, reading, writing, and voluntary movement.
• The cerebellum, which is in the lower back of the brain (near the middle of the back of the head), controls movement, balance, and posture.
• The brain stem connects the brain to the spinal cord. It is in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.

The spinal cord connects the brain with nerves in most parts of the body.

The spinal cord is a column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. These membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain about the sense of touch.

Brain and spinal cord tumors are a common type of childhood cancer.

Although cancer is rare in children, brain and spinal cord tumors are the third most common type of childhood cancer, after leukemia and lymphoma. Brain tumors can occur in both children and adults. Treatment for children is usually different than treatment for adults. (See the PDQ treatment summary on Adult Brain Tumors ¹ for more information.)

This summary describes the treatment of primary brain and spinal cord tumors (tumors that begin in the brain and spinal cord). Treatment of metastatic brain and spinal cord tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain or spinal cord, is not covered in this summary.

There are different types of childhood brain and spinal cord tumors.

Childhood brain and spinal cord tumors are named based on the type of cell they formed in and where the tumor first formed in the CNS.

Astrocytomas

Childhood astrocytomas are tumors that form in cells called astrocytes. They can be high-grade or low-grade tumors. The grade of the tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. High-grade astrocytomas are fast-growing, malignant tumors. Low-grade astrocytomas are slow-growing tumors that are less likely to be malignant. (See the PDQ summaries on Childhood Cerebellar Astrocytoma Treatment ², Childhood Cerebral Astrocytoma/Malignant Glioma Treatment ³, and Childhood Visual Pathway and Hypothalamic Glioma Treatment ⁴ for more information.)

Atypical Teratoid/Rhabdoid Tumor

Childhood atypical teratoid/rhabdoid tumors are fast-growing tumors that often form in the cerebellum. They may also form in other parts of the brain and in the spinal cord. (See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment ⁵ for more information.)

Brain Stem Glioma

Childhood brain stem gliomas form in the brain stem (the part of the brain connected to the spinal cord). (See the PDQ summary on Childhood Brain Stem Glioma Treatment ⁶ for more information.)

Central Nervous System Embryonal Tumor

Childhood CNS embryonal tumors form in brain and spinal cord cells when the fetus is beginning to develop. They include the following types of tumors:

• CNS atypical teratoid/rhabdoid tumors (See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment ⁵ for more information.)
• Ependymoblastoma
• Medulloblastoma
• Medulloepithelioma
• Pineal parenchymal tumors
• Pineoblastoma
• Supratentorial primitive neuroectodermal tumors (SPNET)

(See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment ⁷ for more information.)

Central Nervous System Germ Cell Tumor

Childhood CNS germ cell tumors form in germ cells, which are cells that develop into sperm or ova (eggs). There are different types of childhood germ cell tumors. These include germinomas, embryonal yolk sac carcinomas, choriocarcinomas, and teratomas. A mixed germ cell tumor has two types of germ cell tumors in it. Germ cell tumors can be either benign or malignant.

Germ cell brain tumors usually form in the center of the brain, near the pineal gland. The pineal gland is a tiny organ in the brain that makes melatonin, which is a substance that helps control the sleeping and waking cycle. Germ cell tumors can spread to other parts of the brain and spinal cord.

Craniopharyngioma

Childhood craniopharyngiomas are tumors that usually form just above the pituitary gland. The pituitary gland is found in the center of the brain behind the back of the nose. It is about the size of a pea and controls many important body functions including growth. Craniopharyngiomas rarely spread, but may affect important areas of the brain, such as the pituitary gland. (See the PDQ summary on Childhood Craniopharyngioma Treatment ⁸ for more information.)

Ependymoma

Childhood ependymomas are slow-growing tumors formed in cells that line the fluid -filled spaces in the brain and spinal cord. (See the PDQ summary on Childhood Ependymoma Treatment ⁹ for more information.)

Medulloblastoma

Childhood medulloblastomas form in the cerebellum. (See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment ⁷ for more information.)

Spinal Cord Tumors

Tumors of many different cell types may form in the spinal cord. Low-grade spinal cord tumors usually do not spread. High-grade spinal cord tumors may spread to other places in the spinal cord or brain.

Supratentorial Primitive Neuroectodermal Tumor

Childhood supratentorial primitive neuroectodermal tumors (SPNET) form in immature cells in the cerebrum. (See the PDQ treatment summary on Childhood Central Nervous System Embryonal Tumors Treatment ⁷ for more information.)

Visual Pathway and Hypothalamic Glioma

Childhood visual pathway and hypothalamic gliomas form in the visual pathway and the hypothalamus of the brain. The visual pathway includes the optic nerve, optic chiasm, and optic tract. The visual pathway sends messages from the eye to the brain and from the brain to the eye. The hypothalamus is an area of the brain that controls body temperature, hunger, and thirst. (See the PDQ summary on Childhood Visual Pathway and Hypothalamic Glioma Treatment ⁴ for more information.)

The cause of most childhood brain and spinal cord tumors is unknown.

The symptoms of childhood brain and spinal cord tumors are not the same in every child.

Headaches and other symptoms may be caused by childhood brain and spinal cord tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

Brain Tumors

• Morning headache or headache that goes away after vomiting.
• Frequent nausea and vomiting.
• Vision, hearing, and speech problems.
• Loss of balance and trouble walking.
• Unusual sleepiness or change in activity level.
• Unusual changes in personality or behavior.
• Seizures.
• Increase in the head size (in infants).

Spinal Cord Tumors

• Back pain or pain that spreads from the back towards the arms or legs.
• A change in bowel habits or trouble urinating.
• Weakness in the legs.
• Trouble walking.

In addition to these symptoms of brain and spinal cord tumors, some children are unable to reach certain growth and development milestones such as sitting up, walking, and talking in sentences.

Tests that examine the brain and spinal cord are used to detect (find) childhood brain and spinal cord 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 person’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.
• Serum tumor marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.
• 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 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).
• 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.
• Angiogram: A procedure to look at blood vessels and the flow of blood in the brain. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages.
• 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 body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

Most childhood brain tumors are diagnosed and removed in surgery.

If doctors think there might be a brain tumor, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle 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 pathologist checks the cancer cells to find out the type and grade of brain tumor. The grade of the tumor is based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread.

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 childhood brain and spinal cord tumors are diagnosed by imaging tests.

Sometimes a biopsy or surgery cannot be done safely because of where the tumor formed in the brain or spinal cord. These tumors are diagnosed based on the results of imaging tests and other procedures ¹⁰.

Certain factors affect prognosis (chance of recovery).

The prognosis (chance of recovery) depends on the following:

• Whether there are any cancer cells left after surgery.
• The type of tumor.
• The location of the tumor.
• The child's age.
• Whether the tumor has just been diagnosed or has recurred (come back).

Glossary Terms

Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).

An x-ray of blood vessels. The person receives an injection of dye to outline the vessels on the x-ray.

A type of protein made by plasma cells (a type of white blood cell) in response to an antigen (foreign substance). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.

A substance that causes the immune system to make a specific immune response.

A large, star-shaped cell that holds nerve cells in place and helps them develop and work the way they should. An astrocyte is a type of glial cell.

A tumor that begins in the brain or spinal cord in small, star-shaped cells called astrocytes.

An aggressive cancer of the central nervous system, kidney, or liver that occurs in very young children. Also called AT/RT and ATT/RHT.

Not cancer. Benign tumors may grow larger but do not spread to other parts of the body.

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.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.

The long, tube-shaped organ in the abdomen that completes the process of digestion. The bowel has two parts, the small bowel and the large bowel. Also called intestine.

The part of the brain that is connected to the spinal cord.

A tumor located in the part of the brain that connects to the spinal cord (the brain stem). It may grow rapidly or slowly, depending on the grade of the tumor.

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also 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.

Cancer that begins in the skin or in tissues that line or cover internal organs.

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

The brain and spinal cord. Also called CNS.

A tumor of the central nervous system, including brain stem glioma, craniopharyngioma, medulloblastoma, and meningioma. Also called CNS tumor.

The portion of the brain in the back of the head between the cerebrum and the brain stem. The cerebellum controls balance for walking and standing, and other complex motor functions.

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 malignant, fast-growing tumor that develops from trophoblastic cells (cells that help an embryo attach to the uterus and help form the placenta). Almost all choriocarcinomas form in the uterus after fertilization of an egg by a sperm, but a small number form in a testis or an ovary. Choriocarcinomas spread through the blood to other organs, especially the lungs. They are a type of gestational trophoblastic disease. Also called chorioblastoma, chorioepithelioma, and chorionic carcinoma.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

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.

A benign brain tumor that may be considered malignant because it can damage the hypothalamus, the area of the brain that controls body temperature, hunger, and thirst.

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

The study of chromosomes and chromosomal abnormalities.

The process of identifying a disease, such as cancer, from its signs and symptoms.

A microscope (device used to magnify small objects) that uses electrons (instead of light) to produce an enlarged image. An electron microscope shows tiny details better than any other type of microscope.

A mass of rapidly growing cells that begins in embryonic (fetal) tissue. Embryonal tumors may be benign or malignant, and include neuroblastomas and Wilms tumors. Also called embryoma.

A type of brain tumor that begins in cells lining the spinal cord central canal (fluid-filled space down the center) or the ventricles (fluid-filled spaces of the brain). Ependymomas may also form in the choroid plexus (tissue in the ventricles that makes cerebrospinal fluid). Also called ependymal tumor.

The developing offspring from 7 to 8 weeks after conception until birth.

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

A metal element that is used in magnetic resonance imaging (MRI) and other imaging methods. It is a contrast agent, which helps show abnormal tissue in the body during imaging with a special machine.

A reproductive cell of the body. Germ cells are egg cells in females and sperm cells in males.

A type of tumor that begins in the cells that give rise to sperm or eggs. Germ cell tumors can occur almost anywhere in the body and can be either benign or malignant.

The most common type of germ cell tumor in the brain.

A cancer of the brain that begins in glial cells (cells that surround and support nerve cells).

A type of sugar; the chief source of energy for living organisms.

The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Grading systems are different for each type of cancer.

When referring to cancerous and precancerous growths, a term used to describe cells that look abnormal under a microscope. These cells are more likely to grow and spread quickly than cells in low-grade cancerous and precancerous growths.

The area of the brain that controls body temperature, hunger, and thirst.

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

A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.

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.

A microscope (device to magnify small objects) in which objects are lit directly by white light.

When referring to cancerous and precancerous growths, a term used to describe cells that look nearly normal under a microscope. These cells are less likely to grow and spread quickly than cells in high-grade cancerous or precancerous growths.

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.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

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.

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

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, NMRI, and nuclear magnetic resonance imaging.

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.

A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.

A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.

The nerve that carries messages from the retina to the brain.

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

The essential or functional elements of 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 procedure in which a small amount of radioactive glucose (sugar) is injected into a vein, and a scanner is used to make detailed, computerized pictures of areas inside the body where the glucose is used. Because cancer cells often use more glucose than normal cells, the pictures can be used to find cancer cells in the body. Also called positron emission tomography scan.

An exam of the body to check for general signs of disease.

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

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

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 main endocrine gland. It produces hormones that control other glands and many body functions, especially growth.

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.

Giving off radiation.

An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.

To come back or to return.

In medicine, an instrument that takes pictures of the inside of the body.

Convulsion; a sudden, involuntary movement of the muscles.

A blood test that measures the amount of substances called tumor markers (or biomarkers). Tumor markers are released into the blood by tumor cells or by other cells in response to tumor cells. A high level of a tumor marker may be a sign of cancer.

The male reproductive cell, formed in the testicle. A sperm unites with an egg to form an embryo.

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 type of germ cell tumor that may contain several different types of tissue, such as hair, muscle, and bone. Teratomas occur most often in the ovaries in women, the testicles in men, and the tailbone in children. Not all teratomas are malignant.

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

A substance that may be found in tumor tissue or released from a tumor into the blood or other body fluids. A high level of a tumor marker may mean that a certain type of cancer is in the body. Examples of tumor markers include CA 125 (in ovarian cancer), CA 15-3 (in breast cancer), CEA (in ovarian, lung, breast, pancreas, and gastrointestinal tract cancers), and PSA (in prostate cancer).

Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.

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

A rare, slow-growing tumor that usually forms in the optic nerve, optic chiasm, or optic tract. These are parts of the nervous system that carry messages from the eye to the brain. Also called optic pathway glioma.

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.