21st Century Pediatric Cancer Sourcebook: Childhood Brain Stem Glioma. Pontine Glioma - Clinical Data and Practical Information for Patients, Families, and Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
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PART ONE
Chapter 1A: Childhood Brain Stem Glioma
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
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Chapter 1A: Childhood Brain Stem Glioma
Last Modified: 01/06/2011
Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem.
The cause of most childhood brain tumors is unknown.
The symptoms of childhood brain stem glioma vary and often depend on the child’s age and where the tumor is located.
Tests that examine the brain are used to detect (find) childhood brain stem glioma.
Some childhood brain stem gliomas are diagnosed and removed in surgery.
Certain factors affect prognosis (chance of recovery) and treatment options.
Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem.
The brain stem is the part of the brain connected to the spinal cord. It is located in the lowest part of the brain, just above the back of the neck. The brain stem is the part of the brain that controls breathing, heart rate, and nerves and muscles used in seeing, hearing, walking, talking, and eating. Most childhood brain stem gliomas are pontine gliomas, which form in a part of the brain stem called the pons. Brain tumors are the third most common type of cancer in children.
Anatomy of the brain, showing the cerebrum, cerebellum, brain stem, and other parts of the brain. Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.
The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause symptoms and need treatment.
This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults.
The cause of most childhood brain tumors is unknown.
The symptoms of childhood brain stem glioma vary and often depend on the child’s age and where the tumor is located.
The following symptoms and others may be caused by a brain stem glioma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur:
Loss of balance and trouble walking.
Vision and hearing problems.
Morning headache or headache that goes away after vomiting.
Nausea and vomiting.
Unusual sleepiness or change in energy level.
Tests that examine the brain are used to detect (find) childhood brain stem glioma.
The following imaging tests may be used:
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Some childhood brain stem gliomas are diagnosed and removed in surgery.
If the tumor has not spread widely within the brain stem or has not been diagnosed by MRI, a biopsy may be 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.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
The type of brain stem glioma.
Where the tumor is found in the brain and if it has spread within the brain stem.
Whether or not the child has a condition called neurofibromatosis type.
Whether the tumor has just been diagnosed or has recurred (come back).
Stages of Childhood Brain Stem Glioma
Key Points for This Section
The plan for cancer treatment depends on whether the tumor is in one area of the brain or has spread throughout the brain.
There are three ways that cancer spreads in the body.
The information from tests and procedures done to detect (find) childhood brain stem glioma is used to plan cancer treatment.
The plan for cancer treatment depends on whether the tumor is in one area of the brain or has spread throughout the brain.
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 brain stem glioma. Instead, the plan for cancer treatment depends on whether the tumor is diffuse (spread throughout the brain) or focal (in one area of the brain):
Diffuse intrinsic pontine glioma is a tumor that has spread widely throughout the brain stem. A biopsy is usually not done for this type of brain stem glioma and it is not removed by surgery. A diffuse intrinsic pontine glioma is usually diagnosed using imaging studies.
Focal or low-grade glioma is a tumor that is in one area of the brain stem. A biopsy may be done and the tumor removed during the same surgery.
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 brain stem glioma is used to plan cancer treatment.
Some of the tests used to detect childhood brain stem glioma are repeated if the tumor is removed by surgery. (See the General Information 4 section.) This is to find out how much tumor remains after surgery and plan further treatment.
Recurrent Childhood Brain Stem Glioma
Recurrent childhood brain stem glioma is a tumor that has recurred (come back) after it has been treated. If childhood brain stem glioma recurs, it may do so many years after initial treatment. The tumor may come back in the brain or in other parts of the central nervous system.
Treatment Option Overview
Key Points for This Section
There are different types of treatment for children with brain stem glioma.
Children with brain stem glioma 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.
Five types of standard treatment are used:
Surgery * Radiation therapy * Chemotherapy * Cerebrospinal fluid diversion * Watchful waiting
New types of treatment are being tested in clinical trials.
Radiation therapy with radiosensitizers
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 brain stem glioma.
Different types of treatment are available for children with brain stem glioma. 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 brain stem glioma 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:
Neurosurgeon. * Neuropathologist. * Radiation oncologist. * Neuro-oncologist. * Neurologist. * Rehabilitation specialist. * Neuroradiologist. * 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 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.
Five types of standard treatment are used:
Surgery
Surgery is used to diagnose and treat childhood brain stem glioma as discussed in the General Information section of this summary.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells 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. Certain ways of giving radiation therapy can help keep radiation from damaging healthy tissue:
Conformal radiation therapy is a type of radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams 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.
Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day.
The way the radiation therapy is given depends on the type and stage of the cancer being treated. Radiation therapy may be used alone or in addition to chemotherapy.
Several months after radiation therapy to the brain, imaging tests may show changes to the brain tissue. These changes may be caused by the radiation therapy or may mean the tumor is growing. It is important to be sure the tumor is growing before planning more treatment.
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 in 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.
Because radiation therapy to the brain can affect growth and brain development in young children, clinical trials are studying ways of using chemotherapy to delay or reduce the need for radiation therapy.
Cerebrospinal fluid diversion
Cerebrospinal fluid diversion is a method used to drain fluid that has built up around the brain and spinal cord. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries excess fluid away from the brain so it may be absorbed elsewhere in the body.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
New types of treatment are being tested in clinical trials.
This summary 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.
Radiation therapy with radiosensitizers
Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor 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.
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 Brain Stem Glioma
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.
Untreated Childhood Brain Stem Glioma
Untreated childhood brain stem glioma is a tumor for which no treatment has been given. The child may have received drugs or treatment to relieve symptoms caused by the tumor.
Standard treatment of diffuse intrinsic pontine glioma is usually radiation therapy.
Some of the treatments being studied in clinical trials for diffuse intrinsic pontine glioma include the following:
Radiation therapy with a radiosensitizer.
New anticancer drugs given at the same time as radiation therapy to the brain stem.
Standard treatment of focal or low-grade glioma may include the following:
Surgery with or without radiation therapy, which may be followed by adjuvant chemotherapy.
Cerebrospinal fluid diversion followed by watchful waiting.
Treatment of brain stem glioma in children with neurofibromatosis type 1 may be watchful waiting. The tumors are slow-growing in these children and may not need specific treatment for years.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood brain stem glioma. 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.
Recurrent Childhood Brain Stem Glioma
Treatment of recurrent childhood brain stem glioma depends on the type of tumor, whether it comes back in the place in which it started or in another part of the brain, and the type of treatment previously given.
Standard treatment of recurrent diffuse intrinsic pontine glioma is usually palliative therapy, to relieve symptoms and improve the patient's quality of life. The patient may also be treated in a clinical trial of a new treatment.
Treatment of recurrent focal or low-grade childhood brain stem glioma may include the following:
Surgery.
Radiation therapy.
Chemotherapy.
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 brain stem glioma. 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
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
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 stem glioma (...glee-OH-muh)
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.
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.
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.
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.
glioma (glee-OH-muh)
A cancer of the brain that begins in glial cells (cells that surround and support nerve cells).
imaging procedure
A method of producing pictures of areas inside the body. Also called imaging test.
injection
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
malignant (muh-LIG-nunt)
Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
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.
neurofibromatosis type 1 (NOOR-oh-FY-broh-muh-TOH-sis tipe 1)
A rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin. Also called NF1.
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.
pons
Part of the central nervous system, located at the base of the brain, between the medulla oblongata and the midbrain. It is part of the brainstem.
pontine (PON-teen)
Having to do with the pons (part of the central nervous system, located at the base of the brain, between the medulla oblongata and the midbrain).
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.
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.
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.
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Childhood Brain Stem Glioma Treatment - Last Modified: 08/06/2010 - Health Professional Version
Cellular Classification * Stage Information * Treatment Option Overview * Untreated Childhood Brain Stem Glioma Diffuse Intrinsic Pontine Gliomas * Treatment options under clinical evaluation * Focal or Low-grade Brain Stem Gliomas * Neurofibromatosis * Current Clinical Trials * Recurrent Childhood Brain Stem Glioma Diffuse Intrinsic Pontine Gliomas * Focal or low-grade brain stem gliomas * Treatment Options Under Clinical Evaluation * Current Clinical Trials
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.
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]
Cellular Classification
Brain stem gliomas are classified according to their location, radiographic appearance, and histology (when obtained). Brain stem gliomas may occur in the pons, midbrain, tectum, dorsum of the medulla at the cervicomedullary junction, or in multiple regions of the brain stem. The tumor may contiguously involve the cerebellar peduncles, cerebellum, the cervical spinal cord, and/or thalamus. The majority of childhood brain stem gliomas are diffuse, fibrillary astrocytomas that involve the pons (diffuse intrinsic pontine gliomas [DIPG]), often with contiguous involvement of other brain stem sites.[1,2] The prognosis is extremely poor for these tumors. The biology of diffuse pontine glioma is limited by the lack of pathological tissue available for study, but this is slowly being remedied by occasional upfront biopsies or analysis of immediate postmortem autopsy specimens. Overexpression of epidermal growth factor receptor, platelet-derived growth factor receptor alpha, and poly (ADP-ribose) polymerase (PARP-1) have been identified.[3,4] Focal pilocytic astrocytomas have a more favorable prognosis. These most frequently arise in the tectum of the midbrain, focally, within the pons, or at the cervicomedullary junction where they are often exophytic, and have a far better prognosis than diffuse intrinsic tumors.[5-7]
Primary tumors of the brain stem are most often diagnosed based on clinical findings and on neuroimaging studies using magnetic resonance imaging.[8,9] Histologic confirmation of presumed DIPG is usually unnecessary. Biopsy or resection may be indicated for brain stem tumors that are not diffuse and intrinsic or when there is diagnostic uncertainty based on imaging findings. New approaches with stereotactic needle biopsy may make biopsy safer.[10]
References
Freeman CR, Farmer JP: Pediatric brain stem gliomas: a review. Int J Radiat Oncol Biol Phys 40 (2): 265-71, 1998. [PUBMED Abstract]
Laigle-Donadey F, Doz F, Delattre JY: Brainstem gliomas in children and adults. Curr Opin Oncol 20 (6): 662-7, 2008. [PUBMED Abstract]
Gilbertson RJ, Hill DA, Hernan R, et al.: ERBB1 is amplified and overexpressed in high-grade diffusely infiltrative pediatric brain stem glioma. Clin Cancer Res 9 (10 Pt 1): 3620-4, 2003. [PUBMED Abstract]
Zarghooni M, Bartels U, Lee E, et al.: Whole-genome profiling of pediatric diffuse intrinsic pontine gliomas highlights platelet-derived growth factor receptor alpha and poly (ADP-ribose) polymerase as potential therapeutic targets. J Clin Oncol 28 (8): 1337-44, 2010. [PUBMED Abstract]
Epstein F, McCleary EL: Intrinsic brain-stem tumors of childhood: surgical indications. J Neurosurg 64 (1): 11-5, 1986. [PUBMED Abstract]
Edwards MS, Wara WM, Ciricillo SF, et al.: Focal brain-stem astrocytomas causing symptoms of involvement of the facial nerve nucleus: long-term survival in six pediatric cases. J Neurosurg 80 (1): 20-5, 1994. [PUBMED Abstract]
Pollack IF, Pang D, Albright AL: The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors. J Neurosurg 80 (4): 681-8, 1994. [PUBMED Abstract]
Albright AL, Packer RJ, Zimmerman R, et al.: Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children's Cancer Group. Neurosurgery 33 (6): 1026-9; discussion 1029-30, 1993. [PUBMED Abstract]
Liu AK, Brandon J, Foreman NK, et al.: Conventional MRI at presentation does not predict clinical response to radiation therapy in children with diffuse pontine glioma. Pediatr Radiol 39 (12): 1317-20, 2009. [PUBMED Abstract]
Cartmill M, Punt J: Diffuse brain stem glioma. A review of stereotactic biopsies. Childs Nerv Syst 15 (5): 235-7; discussion 238, 1999. [PUBMED Abstract]
Stage Information
There is no generally applied staging system for childhood brain stem gliomas.[1] It is uncommon for these tumors to have spread outside the brain stem itself at the time of initial diagnosis. Spread of malignant brain stem tumors is usually contiguous; metastasis via the subarachnoid space has been reported in up to 30% of cases diagnosed antemortem.[2] Such dissemination may occur prior to local relapse but usually occurs simultaneously with or after local disease relapse.
The less common tumors of the midbrain, especially in the tectal plate region, have been viewed separately from those of the brain stem because they are more likely to be low grade and have a greater likelihood of long-term survival (approximately 80% 5-year progression-free survival vs. <10% for tumors of the pons).[1,3-7] Similarly, dorsally exophytic and cervicomedullary tumors are generally low grade and have a relatively favorable prognosis. Children younger than 3 years may have a more favorable prognosis, perhaps reflecting different biologic characteristics.[8]
References
Freeman CR, Farmer JP: Pediatric brain stem gliomas: a review. Int J Radiat Oncol Biol Phys 40 (2): 265-71, 1998. [PUBMED Abstract]
Packer RJ, Allen J, Nielsen S, et al.: Brainstem glioma: clinical manifestations of meningeal gliomatosis. Ann Neurol 14 (2): 177-82, 1983. [PUBMED Abstract]
Halperin EC, Wehn SM, Scott JW, et al.: Selection of a management strategy for pediatric brainstem tumors. Med Pediatr Oncol 17 (2): 117-26, 1989. [PUBMED Abstract]
Epstein F, McCleary EL: Intrinsic brain-stem tumors of childhood: surgical indications. J Neurosurg 64 (1): 11-5, 1986. [PUBMED Abstract]
Edwards MS, Wara WM, Ciricillo SF, et al.: Focal brain-stem astrocytomas causing symptoms of involvement of the facial nerve nucleus: long-term survival in six pediatric cases. J Neurosurg 80 (1): 20-5, 1994. [PUBMED Abstract]
Pollack IF, Pang D, Albright AL: The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors. J Neurosurg 80 (4): 681-8, 1994. [PUBMED Abstract]
Mandell LR, Kadota R, Freeman C, et al.: There is no role for hyperfractionated radiotherapy in the management of children with newly diagnosed diffuse intrinsic brainstem tumors: results of a Pediatric Oncology Group phase III trial comparing conventional vs. hyperfractionated radiotherapy. Int J Radiat Oncol Biol Phys 43 (5): 959-64, 1999. [PUBMED Abstract]
Broniscer A, Laningham FH, Sanders RP, et al.: Young age may predict a better outcome for children with diffuse pontine glioma. Cancer 113 (3): 566-72, 2008. [PUBMED Abstract]
Treatment Option Overview
Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best available, accepted therapy. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those that were previously obtained with existing therapy.
Because of the relative rarity of cancer in children, all patients with brain tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy (including three-dimensional conformal radiation therapy) of pediatric brain tumors is technically very demanding and should be carried out in centers that have experience in that area in order to ensure optimal results.
Untreated Childhood Brain Stem Glioma
Note: Some citations in the text of this section are followed by a level of evidence.