21st Century Pediatric Cancer Sourcebook: Childhood Ependymoma, Subependymoma - Clinical Data and Practical Information for Patients, Families, and Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
Questions? Suggestions? Comments? Concerns? Please contact the publisher directly at
Remember, the book retailer can't answer your questions, but we can!
* * * * * * * * * * *
Smashwords Edition, License Notes
This ebook is licensed for your personal enjoyment only. This ebook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each person you share it with. If you're reading this book and did not purchase it, or it was not purchased for your use only, then you should return to Smashwords.com and purchase your own copy. Thank you for respecting the hard work of this author.
* * * * * * * * * * * *
IMPORTANT NOTE: Information in this e-book is not a substitute for professional medical advice. If you have or suspect that you have any illness, you must consult with a physician or professional healthcare provider! Call 911 and get to the nearest emergency room if you have serious or worsening symptoms.
This material represents a snapshot in time, with authoritative information formatted for ebook reading that was up-to-date at the moment of publication. For the latest cancer updates, please be sure to visit the NCI website:
From our Guide to Leading Medical Websites, here are three valuable sites with authoritative cancer information:
OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
* * * * * * * * * * * *
This is a privately authored news service and educational publication of Progressive Management. Our publications synthesize official government information with original material - they are not produced by the federal government. They are designed to provide a convenient user-friendly reference work to uniformly present authoritative knowledge that can be rapidly read, reviewed or searched. Vast archives of important data that might otherwise remain inaccessible are available for instant review no matter where you are. This e-book format makes a great reference work and educational tool. There is no other reference book that is as convenient, comprehensive, thoroughly researched, and portable - everything you need to know, from renowned experts you trust. For over a quarter of a century, our news, educational, technical, scientific, and medical publications have made unique and valuable references accessible to all people. Our e-books put knowledge at your fingertips, and an expert in your pocket!
PART ONE
Chapter 1A: Childhood Ependymoma
PART TWO
Chapter 1B Late Effects of Treatment for Childhood Cancer - Patient Version
Chapter 2B: Pediatric Supportive Care
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: When Someone You Love Is Being Treated For Cancer
Chapter 15B: Living Beyond Cancer: Finding a New Balance
Chapter 16B: Caring for the Caregiver
Chapter 17B: Young People With Cancer, A Handbook For Parents
Chapter 18B: When Cancer Returns
Chapter 19B: When Someone You Love Has Advanced Cancer / Support for Caregivers
Chapter 20B: Chemotherapy and You
Chapter 21B: Managing Chemotherapy Side Effects - Anemia
Chapter 22B: Managing Chemotherapy Side Effects - Appetite Changes
Chapter 23B: Managing Chemotherapy Side Effects - Bleeding Problems
Chapter 24B: Managing Chemotherapy Side Effects - Constipation
Chapter 25B: Managing Chemotherapy Side Effects - Memory Changes
Chapter 26B: Managing Chemotherapy Side Effects - Mouth and Throat Changes
Chapter 27B: Managing Chemotherapy Side Effects - Nerve Changes
Chapter 28B: Managing Chemotherapy Side Effects - Pain
Chapter 29B: Managing Chemotherapy Side Effects - Skin and Nail Changes
Chapter 30B: Managing Chemotherapy Side Effects - Swelling (Fluid retention)
Chapter 31B: Targeted Cancer Therapies
Chapter 33B : Follow-up Care After Cancer Treatment
Chapter 34B: Radiation Therapy and You
Chapter 36B: Understanding Radiation Therapy, What To Know About External Beam Radiation Therapy
Chapter 37B: Radiation Therapy for Cancer
Chapter 38B: Managing Radiation Therapy Side Effects - What To Do When Your Mouth or Throat Hurts
Chapter 39B: What To Do About Hair Loss (Alopecia)
Chapter 42B: Managing Radiation Therapy Side Effects - Changes When You Urinate
Chapter 43B: Managing Radiation Therapy Side Effects What To Do About Mild Skin Changes
Chapter 45B: General Cancer Information And Resources
Chapter 46B: Cancer And The Environment - What You Need to Know, What You Can Do
Chapter 48B: FDA Warning: Beware of Online Cancer Fraud
Chapter 49B: FDA Office of Oncology Drug Products
Chapter 50B: Understanding the HIPAA Privacy Rule
* * * * * * * * * * * *
Chapter 1A: Childhood Ependymoma
Last Modified: 01/06/2011
Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord.
The central nervous system controls many important body functions.
The cause of most childhood brain tumors is unknown.
The symptoms of childhood ependymoma vary and often depend on the child’s age and where the tumor is located.
Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.
Childhood ependymoma is diagnosed and removed in surgery.
Certain factors affect prognosis (chance of recovery) and treatment options.
The brain controls vital functions such as memory and learning, the senses (hearing, sight, smell, taste, and touch), and emotion. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body.
About 1 in 11 childhood brain tumors are ependymomas. 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.
Ependymomas most commonly form in these parts of the central nervous system (CNS):
Cerebrum: The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement.
Cerebellum: The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture.
Brain stem: The part that connects the brain to the spinal cord, 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.
Spinal cord: The 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. The spinal cord and 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 for the sense of touch.
The cause of most childhood brain tumors is unknown.
The symptoms of childhood ependymoma vary and often depend on the child’s age and where the tumor is located.
The following symptoms and others may be caused by childhood ependymoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur:
Frequent headaches.
Seizures.
Frequent nausea and vomiting.
Loss of balance or trouble walking.
Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.
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.
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 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 to check for cancer cells. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
Childhood ependymoma is 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 the 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. An MRI may be done after the tumor is removed to find out how much tumor remains.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
Whether cancer cells remain after surgery.
The type of ependymoma and whether it begins in the brain or in the spinal cord.
The age of the child when the tumor is diagnosed.
Whether the cancer has spread to other parts of the central nervous system, such as the meninges (membranes covering the brain) and the 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).
Stages of Childhood Ependymoma
Key Points for This Section
The area where the tumor is found and the child’s age are used in place of a staging system to plan cancer treatment.
There are three ways that cancer spreads in the body.
The information from tests and procedures done to detect (find) childhood ependymoma is used to plan cancer treatment.
The area where the tumor is found and the child’s age are used in place of a staging system to plan cancer treatment.
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 ependymoma. Instead, the plan for cancer treatment after surgery depends on the following:
Whether any cancer cells remain after surgery.
Whether the cancer has spread to other parts of the brain or spinal cord.
The age of the child.
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 ependymoma is used to plan cancer treatment.
Some of the tests used to detect childhood ependymoma are repeated after the tumor is removed by surgery. (See the General Information 4 section.) This is to find out how much tumor remains after surgery. Another procedure that may be done to find out if cancer has spread is a lumbar puncture. A lumbar puncture is 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.
Recurrent Childhood Ependymoma
Recurrent childhood ependymoma is a tumor that has recurred (come back) after it has been treated. Childhood ependymoma commonly recurs, usually at the original cancer site. The tumor may come back as long as 15 years or more after initial treatment.
Treatment Option Overview
Key Points for This Section
There are different types of treatment for children with ependymoma.
Children with ependymoma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors.
Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.
Some cancer treatments cause side effects months or years after treatment has ended.
Three types of standard treatment are used:
Surgery
Radiation therapy
Chemotherapy
New types of treatment are being tested in clinical trials.
Watchful waiting
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with ependymoma.
Different types of treatment are available for children with ependymoma. 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 with ependymoma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors.
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:
Pediatric neurosurgeon. * Neurologist. * Neuropathologist. * Neuroradiologist. * Rehabilitation specialist. * Radiation oncologist. * Medical oncologist. * Endocrinologist. * Psychologist.
Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.
Childhood brain and spinal cord tumors may cause symptoms that continue for months or years. Symptoms caused by the tumor may begin before diagnosis. Symptoms caused by treatment may begin during or right after treatment.
Some cancer treatments cause side effects months or years after treatment has ended.
These 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).
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 childhood ependymoma as described in the General Information 6 section of 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. The way the radiation therapy is given depends on the type and stage of the cancer being treated. Childhood ependymoma may be treated with fractionated radiation therapy, which divides the total dose of radiation into several smaller, equal doses delivered over a period of days.
Certain ways of giving radiation therapy can help keep radiation away from healthy tissue:
Conformal radiation therapy uses a computer to create a 3-D picture of the tumor. The radiation beams are shaped to fit the tumor.
Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to kill tumor cells.
Stereotactic radiation therapy uses a head frame attached to the skull to aim radiation beams directly at the tumor.
Radiation therapy to the brain can affect growth and development in young children and is not standard treatment for children younger than 3 years. For this reason, conformal radiation therapy and proton-beam therapy that limit damage to healthy brain tissue are being studied in infants and children with ependymoma.
Damage to the brain in young children treated for ependymoma is not always due to the effects of radiation therapy. For example, when hydrocephalus (abnormal buildup of fluid in the brain) is found at diagnosis, it is linked with lower intelligence test scores following surgery and before 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 cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
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.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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 condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Ependymoma
A link to a list of current clinical trials is included for each treatment section. 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 Ependymoma
Newly diagnosed childhood ependymoma is a tumor that has not been treated. The patient may have received drugs or treatment, however, to relieve symptoms caused by the tumor.
Initial treatment for newly diagnosed childhood ependymoma is usually surgery, with or without additional treatment.
After surgery, treatment depends on the age of the child, the amount of tumor that was removed, and whether cancer cells have spread to other parts of the central nervous system.
When the tumor is completely removed by surgery and cancer cells have not spread within the central nervous system, treatment may include the following:
Radiation therapy to the tumor bed (where the tumor was before it was removed), for children aged 3 years or older.
Chemotherapy, for children younger than 3 years.
A clinical trial of watchful waiting, for children aged 1 year or older.
A clinical trial of conformal radiation therapy.
A clinical trial of chemotherapy with or without radiation therapy.
When a part of the tumor remains after surgery, but cancer cells have not spread within the central nervous system, treatment may include the following:
Second-look surgery.
Radiation therapy to the tumor bed, for children aged 3 years or older.
A clinical trial of chemotherapy followed by second-look surgery and conformal radiation therapy.
When cancer cells have spread within the central nervous system, treatment may include the following:
Radiation therapy to the whole brain and spine.
A clinical trial of radiation therapy and chemotherapy.
Recurrent Childhood Ependymoma
Treatment of recurrent childhood ependymomas may include the following:
Surgery.
Radiation therapy, including stereotactic radiation therapy.
Chemotherapy, if chemotherapy was not used when the tumor was first diagnosed.
A clinical trial of a new treatment.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood ependymoma. 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.
Glossary Terms
biopsy (BY-op-see)
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.
brain stem
The part of the brain that is connected to the spinal cord.
brain tumor
The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (not cancer) or malignant (cancer).
cancer (KAN-ser)
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 a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a 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. Also called malignancy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
central nervous system (SEN-trul NER-vus SIS-tem)
The brain and spinal cord. Also called CNS.
cerebellum (ser-uh-BEL-um)
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.
cerebrospinal fluid (seh-REE-broh-SPY-nul FLOO-id)
The fluid that flows in and around the hollow spaces of the brain and spinal cord, and between two of the meninges (the thin layers of tissue that cover and protect the brain and spinal cord). Cerebrospinal fluid is made by tissue called the choroid plexus in the ventricles (hollow spaces) in the brain. Also called CSF.
cerebrum (seh-REE-brum)
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.
contrast material
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.
CT 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 CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
ependymoma (eh-PEN-dih-MOH-muh)
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.
fiber (FY-ber)
In food, fiber is the part of fruits, vegetables, legumes, and whole grains that cannot be digested. The fiber in food may help prevent cancer. In the body, fiber refers to tissue made of long threadlike cells, such as muscle fiber or nerve fiber.
gadolinium texaphyrin (GA-doh-LIH-nee-um tek-SA-fih-rin)
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.
injection
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
leukemia (loo-KEE-mee-uh)
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.
lumbar puncture (LUM-bar PUNK-cher)
A procedure in which a thin needle called a spinal needle is put into the lower part of the spinal column to collect cerebrospinal fluid or to give drugs. Also called spinal tap.
lung
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
lymphoma (lim-FOH-muh)
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.
membrane (MEM-brayn)
A very thin layer of tissue that covers a surface.
meninges (meh-NIN-jees)
The three thin layers of tissue that cover and protect the brain and spinal cord.
metastatic (meh-tuh-STA-tik)
Having to do with metastasis, which is the spread of cancer from the primary site (place where it started) to other places in the body.
MRI
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.
nausea
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.
nerve
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.
neurological exam (NOOR-oh-LAH-jih-kul eg-ZAM)
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.
organ
A part of the body that performs a specific function. For example, the heart is an organ.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
primary tumor (PRY-mayr-ee TOO-mer)
The original tumor.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
recur
To come back or to return.
seizure (SEE-zhur)
Convulsion; a sudden, involuntary movement of the muscles.
spinal cord (SPY-nul kord)
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.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
tissue (TISH-oo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
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.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
vertebral column
The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The vertebral column encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and spine.
vital
Necessary to maintain life. Breathing is a vital function.
vomit
To eject some or all of the contents of the stomach through the mouth.
x-ray
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.
* * * * * * * * * * * *
Childhood Ependymoma Treatment - Last Modified: 08/06/2010 - Health Professional Version
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2002, childhood cancer mortality has decreased by more than 50%.[1] Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment.
Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.
Incidence and Molecular Determinants
Childhood ependymoma comprises approximately 9% of all childhood brain tumors representing approximately 200 cases per year in the United States.[2,3]
Molecular determinants of outcome for ependymomas are just being identified. Studies have identified numerous chromosomal aberrations and related molecular genetic changes. Gain of 1q25, overexpression of EGFR, hTERT expression, high levels of nucleolin, activation of the Notch pathway or Tenascin C, and others have been related to poorer prognosis.[4-10]
References
Smith MA, Seibel NL, Altekruse SF, et al.: Outcomes for children and adolescents with cancer: challenges for the twenty-first century. J Clin Oncol 28 (15): 2625-34, 2010. [PUBMED Abstract]
Gurney JG, Smith MA, Bunin GR: CNS and miscellaneous intracranial and intraspinal neoplasms. In: Ries LA, Smith MA, Gurney JG, et al., eds.: Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. Bethesda, Md: National Cancer Institute, SEER Program, 1999. NIH Pub.No. 99-4649., Chapter 3, pp 51-63. Also available online 3. Last accessed July 14, 2009.
Central Brain Tumor Registry of the United States.: Statistical Report: Primary Brain Tumors in the United States, 1997-2001. Hinsdale, Ill: Central Brain Tumor Registry of the United States, 2004. Also available online 4. Last accessed July 14, 2009.
Tabori U, Ma J, Carter M, et al.: Human telomere reverse transcriptase expression predicts progression and survival in pediatric intracranial ependymoma. J Clin Oncol 24 (10): 1522-8, 2006. [PUBMED Abstract]
Mendrzyk F, Korshunov A, Benner A, et al.: Identification of gains on 1q and epidermal growth factor receptor overexpression as independent prognostic markers in intracranial ependymoma. Clin Cancer Res 12 (7 Pt 1): 2070-9, 2006. [PUBMED Abstract]
Pezzolo A, Capra V, Raso A, et al.: Identification of novel chromosomal abnormalities and prognostic cytogenetics markers in intracranial pediatric ependymoma. Cancer Lett 261 (2): 235-43, 2008. [PUBMED Abstract]
Preusser M, Heinzl H, Gelpi E, et al.: Ki67 index in intracranial ependymoma: a promising histopathological candidate biomarker. Histopathology 53 (1): 39-47, 2008. [PUBMED Abstract]
Tabori U, Wong V, Ma J, et al.: Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma. Br J Cancer 99 (7): 1129-35, 2008. [PUBMED Abstract]
Puget S, Grill J, Valent A, et al.: Candidate genes on chromosome 9q33-34 involved in the progression of childhood ependymomas. J Clin Oncol 27 (11): 1884-92, 2009. [PUBMED Abstract]
Ridley L, Rahman R, Brundler MA, et al.: Multifactorial analysis of predictors of outcome in pediatric intracranial ependymoma. Neuro Oncol 10 (5): 675-89, 2008. [PUBMED Abstract]
Histopathologic Classification of Childhood Ependymal Tumors
In the most recent World Health Organization (WHO) classification of brain tumors, ependymal tumors are classified into four main subtypes:[1]
Subependymoma (WHO Grade I).
Myxopapillary ependymoma (WHO Grade I).
Ependymoma (WHO Grade II). Variants include cellular, papillary, tanycytic, clear cell, and mixed.
Anaplastic (also known as malignant) ependymoma (WHO Grade III).
The subependymoma is a slow-growing benign neoplasm, typically attached to the ventricle wall, and is composed of glial tumor cell clusters embedded in a fibrillary matrix. The myxopapillary ependymoma arises almost exclusively in the location of the conus medullaris, cauda equina, and filum terminale of the spinal cord, and is characterized histologically by tumor cells arranged in a papillary manner around vascularized myxoid stromal cores.
The ependymoma, which is considered a Grade II neoplasm originating from the walls of the ventricles or from the spinal canal, is composed of neoplastic ependymal cells. Ependymomas are subdivided, based on histological findings, into four subtypes: