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Quarta-feira, 17.06.15

Brain Stem Glioma - Childhood - Overview ASCO 2015 - 1

Brain Stem Glioma - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Childhood Brain Stem Glioma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About the brain stem

The brain stem connects the brain to the spinal cord. It is the lowest portion of the brain, located above the back of the neck. The brain stem controls many of the body’s basic functions, such as motor skills, sensory activity, coordination and walking, the beating of the heart, and breathing. It has three parts:

  • The midbrain, which develops from the middle of the brain
  • The medulla oblongata, which connects to the spinal cord
  • The pons, which is located between the medulla oblongata and the midbrain

About brain stem glioma

Brain stem glioma is a type of central nervous system (CNS; brain and spinal cord) tumor that begins when healthy cells in the brain stem change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread. A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain.

Usually, by the time brain stem glioma is diagnosed, it is most often diffuse, which means it has spread freely through the brain stem. This type of tumor is typically very aggressive, meaning that it grows and spreads quickly. A small percentage of brain stem tumors are very localized, called focal tumors. A focal tumor is often less likely to grow and spread quickly.

Brain stem glioma occurs most commonly in children between five and 10 years old. Most brain stem tumors develop in the pons and grow in a part of the brain stem where it can be difficult to perform surgery, making brain stem glioma challenging to treat (see the Treatment Options section).

This section covers brain stem glioma diagnosed in children. Read more about brain tumors in adults.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items. Please note these links will take you to other sections on Cancer.Net:

The next section in this guide is Statistics and it helps explain how many children are diagnosed with this disease and general survival rates. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Brain Stem Glioma - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will find information about how many children are diagnosed with brain stem glioma each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

Approximately 4,000 CNS tumors are diagnosed each year in children younger than 20. Brain stem tumors account for 10% of all childhood brain tumors. After leukemia, CNS tumors are the second most common childhood cancer, accounting for about 26% of cancer in children younger than 15.

The survival rate is the percentage of people who survive after the tumor is found. The survival rate for children with brain stem glioma varies depending on the location of the tumor. Long-term survival rates for children with a tumor in the midbrain or the medulla oblongata range from 65% to 90%. However, a pontine glioma, which is a tumor located in the pons, is more difficult to treat and often worsens quickly. It is uncommon for a child with a tumor in this location to live longer than 12 to 14 months after diagnosis.

Survival statistics should be interpreted with caution. Estimates are based on data from thousands of children with this type of tumor, so the actual risk for a particular individual may be different. It is not possible to tell a person how long he or she will live with a brain stem glioma. Because the survival statistics are measured in multi-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's publication, Cancer Facts and Figures 2015 and St. Jude Children’s Research Hospital.

The next section in this guide is Medical Illustrations and it offers drawings of body parts often affected by this disease. Or, use the menu on the left side of your screen to choose another section to continue reading this guide.  

 

Brain Stem Glioma - Childhood - Medical Illustrations

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will find a basic drawing about the main body parts affected by this disease. To see other pages, use the menu on the side of your screen.

Brain Stem Glioma Anatomy

Larger image

To continue reading this guide, use the menu on the side of your screen to select another section.  

The next section in this guide is Risk Factors and it explains what factors may increase the chance of developing this disease. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Brain Stem Glioma - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will find out more about the factors that increase the chance of developing brain stem glioma. To see other pages in this guide, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do.

Doctors and researchers don’t know what causes most childhood tumors, including brain stem glioma. There is some evidence that genetic factors may play a role in a small percentage of brain stem gliomas.

The following genetic conditions are associated with a higher risk of developing a CNS tumor:

The next section in this guide is Symptoms and Signs and it explains what body changes or medical problems this disease can cause. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Brain Stem Glioma - Childhood - Symptoms and signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

Children with a brain stem glioma may experience the following symptoms or signs. Sometimes, children with a brain stem glioma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a brain stem glioma.          

  • Double vision or not being able to close the eyelids
  • Drooping of the face
  • Difficulty chewing and swallowing food
  • Weakness in the arms and legs, clumsiness or wobbliness, and difficulty walking
  • Difficulty talking
  • Headache
  • Vomiting

If you are concerned about one or more of the symptoms or signs on this list, please talk with your child’s doctor. The doctor will ask how long and how often your child has been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If brain stem glioma is diagnosed, relieving symptoms remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with the health care team about the symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis and it explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Brain Stem Glioma - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a brain stem glioma and find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most other types of tumors, a biopsy is the only way to make a definitive diagnosis. In general, a biopsy is avoided in children with diffuse brain stem glioma because the results of the biopsy do not change treatment options. In addition, the procedure can have serious risks. However, a biopsy may be used when a brain stem glioma has unusual features. As new treatments based on molecular information from the tumor increase and the risk of a biopsy decreases, these procedures may be done more often.

For most patients, diagnosing a brain stem glioma is done with magnetic resonance imaging (MRI) only (see below). Because of this, diffuse brain stem glioma is unlike most other tumors. For a focal tumor, a biopsy and removing the tumor with surgery may be considered. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Other imaging tests may be used to find out whether the tumor has spread.

This list describes options for diagnosing brain stem glioma, and not all tests listed will be used for every person. Your child’s doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

In addition to a physical examination, the following tests may be used to diagnose a brain stem glioma:

  • MRI. An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow.
  • Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Sometimes, a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow. For a brain stem glioma, this test generally does not provide enough information to make a definite diagnosis, and an MRI is still needed.
  • Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. A biopsy is generally not done for the more common, diffuse type of brain stem tumor. However, for a focal tumor, it is often used to find out the type of tumor. If possible, a doctor called a neurosurgeon will remove a small piece of tissue from the brain. A neurosurgeon specializes in treating a CNS tumor using surgery. A pathologist then analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

After diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is brain stem glioma, these results also help the doctor describe the tumor; this is called staging and grading.

The next section in this guide is Stages and Grades, and it explains the system doctors use to describe the extent of the disease. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Brain Stem Glioma - Childhood - Stages and Grades

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will learn about how doctors describe the growth or spread of brain stem glioma. This is called the stage or grade. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of tumors.

There is no formal staging system for childhood brain stem glioma. A tumor may be classified as either diffuse or focal. In addition, the tumor may be classified by its grade.

Grade

Grade describes how much tumor cells look like healthy cells when viewed under a microscope. The doctor compares the tumor’s tissue with healthy tissue. Healthy tissue usually contains many different types of cells grouped together. If the tumor cells looks similar to healthy tissue and contains different cell groupings, it is called differentiated or a low-grade tumor. If the tumor tissue looks very different from healthy tissue, it is called poorly differentiated or a high-grade tumor. The tumor’s grade may help the doctor predict how quickly it will spread. In general, the lower the tumor’s grade, the better the prognosis.

Below are the general classifications for brain stem glioma:

  • Diffuse brain stem glioma. This type of tumor spreads freely throughout the pons and often spreads to the midbrain, the medulla, or nearby parts of the brain. These tend to be high-grade tumors; they are very aggressive and contain abnormal-looking cells.
  • Focal brain stem glioma. About 20% of brain stem tumors are focal, meaning they occur in one area or are contained within a small portion of the brain stem. They usually occur in the midbrain or medulla, rather than the pons. These are usually benign or low-grade tumors; they are less aggressive and the tumor cells look fairly healthy.
  • Recurrent brain stem glioma: Recurrent brain stem glioma is a tumor that has come back after treatment. If the tumor does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the originaldiagnosis.

Information about the tumor’s grade will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Brain Stem Glioma - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/2015

ON THIS PAGE: You will learn about the different ways doctors use to treat children with brain stem glioma. To see other pages, use the menu on the side of your screen.

In general, tumors in children are uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best known treatments available) with newer approaches to treatments that may be more effective. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Studying new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, children should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. For brain stem glioma, a neuro-oncologist may also be involved with treatment. A neuro-oncologist is a doctor who specializes in CNS tumors. If a pediatric cancer center is not nearby, general cancer centers sometimes have pediatric specialists who are able to be part of your child’s care.

In many cases, a team of doctors works with a child and the family to provide care; this is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, dietitians, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Descriptions of the most common treatment options for brain stem glioma are listed below. Treatment options and recommendations depend on several factors, including the type and grade of the tumor, possible side effects, the family’s preferences, and the child’s overall health. Your child’s care plan may also include treatment for symptoms and side effects, an important part of care. Three types of treatments are used to treat brain stem glioma in children: radiation therapy, chemotherapy, and surgery. Sometimes, these treatments are used together.

The treatment of brain stem glioma for children with the genetic condition neurofibromatosis type 1 may differ. A tumor in a child with NF1 may be low-grade even though it looks diffuse. Therefore, active surveillance or watchful waiting may be recommended to watch the tumor for signs that it is worsening. Treatment would begin if the tumor started to grow and spread.

Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with the doctor and what you can expect during the treatment. Learn more about making treatment decisions

Radiation therapy

Radiation therapy is the most common treatment for children with brain stem glioma. Radiation therapy is the use of high-energy x-rays or other particles to destroy tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation therapy is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Because radiation therapy can sometimes cause problems with the growth and development of a child’s brain, the doctor may choose to treat the tumor in another way. To avoid or reduce the need for radiation therapy in young children, the doctor may first use chemotherapy to shrink the tumor. Learn more about the basics of radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to destroy tumor cells, usually by stopping the tumor cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating a tumor with medication, or a pediatric oncologist.

Systemic chemotherapy gets into the bloodstream to reach tumor cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Chemotherapy by itself is not an effective treatment for brain stem glioma. Sometimes, a doctor may use chemotherapy at the same time as or after radiation therapy. The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about the basics of chemotherapy and preparing for treatment. The medications used to treat brain stem glioma are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions by using searchable drug databases.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. A neurosurgeon is a doctor who specializes in treating a CNS tumor using surgery. Surgery is used to treat brain stem glioma only when the tumor looks focal on an MRI scan (see Diagnosis). This means that it may be possible to remove the tumor without damaging the brain, such as when a tumor grows out from the brain stem instead of into the brain stem. For most children with diffuse types of brain stem glioma, surgery is not recommended or possible because of the location of the tumor and the risk involved. Learn more about the basics of surgery.

Getting care for symptoms and side effects

Brain stem glioma and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the tumor, an important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the treatment process. People often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and families report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the tumor, such as chemotherapy, surgery, or radiation therapy. Talk with your child’s doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of your specific treatment plan and palliative care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child is experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care.

Remission and chance of recurrence

A remission is when the tumor cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED. 

A remission may be temporary or permanent. This uncertainty causes many people to worry that the tumor will come back. While many remissions are permanent, it’s important to talk with your child’s doctor about the possibility of the tumor returning. Understanding your child’s risk of recurrence and the treatment options may help you feel more prepared if the disease does return. Learn more about coping with the fear of recurrence.

If the tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your child’s doctor will talk about the treatment options. Often the treatment plan will include the treatments described above such as radiation therapy, chemotherapy, and surgery, but they may be used in a different combination or given at a different pace. Your child’s doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent tumor. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

Treatment for recurrent brain stem glioma depends on the type of tumor, such as whether it is diffuse or focal, and the type of treatment that was given for the original tumor. Depending on the situation, the doctor may recommend either surgery or chemotherapy.

A recurrent tumor may bring up emotions such as disbelief or fear. You and your family are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. Learn more about dealing with a recurrence.

If treatment fails

Although treatment is successful for the majority of children with a tumor, sometimes it is not. If a child’s tumor cannot be cured or controlled, this is called an advanced or terminal tumor. This diagnosis is stressful, and advanced brain stem glioma may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child. Some families find comfort in getting involved in research efforts to advance knowledge about brain stem glioma. Learn more about tissue donation.

The next section in this guide is About Clinical Trials and it offers more information about research studies that are focused on finding better ways to care for people with cancer. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

 

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