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Low Grade Gliomas

  • A low-grade glioma is a slow-growing, less aggressive kind of tumor that can grow in a number of places in the brain and spinal cord. The good news is that, in general, children with low-grade gliomas have better long-term health than those with malignant, high-grade types of pediatric brain tumors.

    More detailed information is available on low-grade gliomas. If you would like to read more general information about brain tumors first, see our overview on childhood brain tumors.

    How Dana-Farber/Boston Children’s approaches low-grade gliomas

    Patients with low-grade gliomas are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Glioma Program, the world’s largest pediatric glioma treatment program.

    After treatment, your child will receive expert follow-up brain tumor survivorship care through the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic, where he will be able to meet with his neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit.

  • What is a pediatric low-grade glioma?

    Low-grade gliomas are a class of slow-growing, less aggressive tumors in the central nervous system. They arise from a type of cell known as a glial cell, and most often a specific kind of glial cell called an astrocyte. Such gliomas are sometimes called astrocytomas.

    Low-grade gliomas are the most common type of brain tumor in children. There are more than 1,000 cases of pediatric low-grade gliomas each year in the United States.

    How are pediatric gliomas classified?

    An important part of diagnosing a pediatric brain tumor involves staging and classifying the disease, which will help your child’s doctor determine treatment options and prognosis. Staging is the process of determining whether the tumor has spread and, if so, how far.

    1. There are four major types of gliomas, classified based on what they look like under a microscope.

    Low grade:

    High grade:

    2. Low-grade gliomas also may be classified according to their location in the brain. These include:

    Are all gliomas astrocytomas?

    No, but most are. Other types of low-grade gliomas include:

    Causes

    What causes low-grade gliomas?

    While research has shown that there is a link between some types of low-grade gliomas and certain genetic diseases (i.e., neurofibromatosis, tuberous sclerosis), these tumors most often occur with no known cause. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

    Symptoms

    What are the symptoms of low-grade gliomas?

    The symptoms of low-grade gliomas can vary greatly depending on the size and location of the tumor and whether it has infiltrated into other areas of the brain or spine.

    Due to the relative slow growth rate of low-grade gliomas, your child’s symptoms may have begun many months or years before they see the doctor. While each child may experience symptoms differently, some of the most common include:

    • change in/loss of vision due to low-grade gliomas of the visual pathway
    • weight gain or loss and/or premature puberty due to low-grade gliomas in the hormone center of the brain
    • problems with movement or bowel/bladder control due to low-grade gliomas in the spine
    • vomiting, headache, fatigue and motor control problems due to fluid build-up in and increased pressure on the brain
    • seizures due to irritation of the normal brain cells

    Keep in mind that these symptoms may resemble other, more common conditions or medical problems.

  • In addition to a physical exam, a medical history and a neurological exam (a test of reflexes, muscle strength, eye and mouth movement, coordination and alertness), your child may require tests, such as:

    • computerized tomography scan (also called a CT or CAT scan) - A diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images of the body. CT scans are more detailed than general x-rays.
    • magnetic resonance imaging (MRI) - A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of the brain and spine.
    • Positron emission tomography - These scans measure the use of glucose (blood sugar) within organs and tissues. Tumors use glucose more quickly than normal tissues, causing them to show up brightly on these scans.
    • biopsy - In many cases, a tissue sample from the tumor is taken through a needle during a simple surgical procedure to confirm the diagnosis. However, with low-grade gliomas of the optic pathway and brain stem, surgery (including biopsy) is generally avoided, due to the very delicate structures in these areas.

    After we complete all necessary tests, our pediatric brain tumor experts meet to review and discuss what they have learned about your child's condition. Then, we will meet with you and your family to discuss the results and outline the best treatment options.

  • Children treated on an outpatient basis for a low-grade glioma at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center are cared for at the Jimmy Fund Clinic on the third floor of the Dana Farber Cancer Institute. If your child needs to be admitted to the hospital, he will stay at Boston Children’s Hospital on the ninth floor of the Berthiaume Building.

    There are a number of treatments that we may recommend for low-grade gliomas. Some of them help to treat the tumor while others are intended to address complications of the disease or side effects of the treatment. The specific course of treatment will be based on several factors, including:

    • your child's age, overall health and medical history
    • type, location and size of the tumor
    • extent of the disease
    • your child's tolerance for specific medications, procedures or therapies
    • how your child's doctors expects the disease to progress

    The treatments available for low-grade gliomas include:

    • Surgery is usually the first step in the treatment of brain tumors. The goal is to remove as much of the tumor as possible without compromising neurological function.
    • Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. Due to the long-term damage that radiation can cause to the developing brain of a child, this treatment is usually only used as a last resort.
    • Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Modern treatments now include biologic (also called smart drugs) that target specific abnormal pathways required by the tumor to grow and spread. A number of these types of drugs are now in clinical trials in children with low-grade gliomas

     Your child may receive chemotherapy:

    • orally, as a pill to swallow
    • intramuscularly (IM), as an injection into the muscle or fat tissue
    • intravenously (IV), directly to the bloodstream
    • intrathecally, with a needle directly into the fluid surrounding the spine

    These treatments may be used alone or in combination. In addition, through the low-grade glioma research program at Dana Farber/Boston Children’s, a number of less toxic biologic targeted therapies for pediatric low-grade gliomas are now available.

    How are side effects of low-grade gliomas managed?

    Side effects in the treatment of low-grade gliomas can arise from surgery, radiation and chemotherapy. Knowing what these side effects are can help you, your child and your care team prepare for, and, in some cases, prevent these symptoms from occurring. 

    • Radiation therapy often produces inflammation, which can make symptoms worse for a short time. To control this, inflammation steroids are sometimes necessary.
    • Chemotherapy drugs cannot tell the difference between normal, healthy cells and cancerous cells. Some of the chemotherapy agents are associated with fatigue, diarrhea, constipation and headache. These side effects can be effectively managed under most circumstances with standard medical approaches.

    Our Glioma Program also provides access to specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and side effects of therapy, include the following:

    • acupuncture/acupressure
    • therapeutic touch
    • massage
    • herbs
    • dietary recommendations

    Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.

    What is expected post-treatment for low-grade glioma?

    The outlook for children with low-grade gliomas can vary significantly depending on:

    • the location of the tumor
    • whether it has spread
    • whether it can be completely surgically removed

    Grade I tumors are usually cured with complete surgical removal. In general, low-grade gliomas have a more positive prognosis than malignant, high-grade brain tumors. The majority of patients are long-term survivors.

    What about progressive or recurrent disease?

    There are numerous standard and experimental treatment options for children with progressive or recurrent low-grade gliomas.

    Resources and support

    There are also a number of patient and family support services at Dana-Farber/Boston Children's to help you and your family through this difficult time.

    Long-term follow-up

    Today, the majority of children and adolescents diagnosed with pediatric brain tumors will survive into adulthood. However, many of them will face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

    To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's Cancer and Blood Disorders Center established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.

    More than 1,000 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multi-disciplinary program designed to address long-term health and social issues for families and survivors of childhood brain tumors. Some of the post-treatment services provided by the Outcomes Clinic include:

    • MRI scans to monitor for tumor recurrences
    • intellectual function evaluation
    • endocrine evaluation and treatment
    • neurologic assessment
    • psychosocial care
    • hearing and vision monitoring
    • ovarian dysfunction evaluation and treatment
    • motor function evaluation and physical therapy
    • complementary medicine

    As a result of treatment, children may experience changes in intellectual and motor function. Among several programs addressing these needs are the School Liaison and Back to School programs, which provide individualized services to ease children's return to school and maximize their ability to learn.

    In addition to providing thorough and compassionate care, our Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families.

    To learn more about our services or to schedule an appointment, call us at 617-632-2680.

  • Dana-Farber/Boston Children's Cancer and Blood Disorders Center is one of nine institutes in the United States belonging to the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group dedicated to the development of new and innovative treatments for children with newly diagnosed oncologic conditions, as well as progressive or recurrent cerebellar astrocytomas and other brain tumors. The consortium offers experimental therapies to patients with relapsed (returning) or refractory (resistant to treatment) disease.

    Dana Farber/Boston Children’s is also home to the Pediatric Low-Grade Astrocytoma (PLGA) Research Program, the world’s largest program dedicated to pediatric low-grade gliomas like cerebellar low-grade astrocytomas, which conducts advanced research on the causes and treatment of low-grade gliomas.

    Through the PLGA program, we have pioneered strategies for analyzing the genetic and molecular characteristics of pediatric low-grade astrocytomas, established a patient registry and multiple international research projects, as well as initiated a number of Phase II protocols using molecular inhibitors for children with progressive/recurrent low-grade gliomas. In collaboration with the Broad Institute of Harvard and MIT, we have made strides toward a better understanding of these conditions.

    Clinical trials

    For many children with brain tumors or other rare or hard-to-treat conditions, clinical trials provide new options.

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