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Anaplastic Astrocytoma

  • Overview

    Astrocytomas are tumors that arise from brain cells called astrocytes, which are a type of glial cell. An anaplastic astrocytoma is a high-grade (malignant) glioma, originating from the glial (supportive) tissue of the brain. Dana-Farber/Children’s Hospital Cancer Center’s Pediatric Brain Tumor Program provides comprehensive treatment and management of children with anaplastic astrocytomas.

    The terms astrocytoma and glioma are sometimes used interchangeably.

    Anaplastic astrocytoma and glioblastoma multiforme, another type of astrocytoma, account for approximately 10 percent of pediatric central nervous system tumors.

    It occurs with increased frequency in families with neurofibromatosis type I, Li-Fraumeni syndrome, hereditary nonpolyposis colon cancer and tuberous sclerosis.

    It is diagnosed using several approaches including CT and MRI imaging scans.

    How Dana-Farber/Children’s Hospital Cancer Center approaches anaplastic astrocytoma

    The usual treatment for anaplastic astrocytomas includes surgery and radiation, and in some cases chemotherapy. The pediatric neuro-oncology and pediatric neurosurgical specialists at Dana-Farber/Children’s Hospital Cancer Center offer:

    • Technological advances such as the intra-operative MRI, which allow our pediatric neurosurgeons to visualize the tumor as they operate with MRI scans, so they can remove as much of the tumor as possible. 
    • Expert diagnosis and consultation by neuropathologists who use advanced molecular diagnostic testing, which may predict which treatments are more likely to work. 
    • Access to unique Phase I clinical trials, from our own investigators, Children’s Oncology Group and the Pediatric Oncology Experimental Therapeutics Consortium, that apply personalized medical approaches, including one investigating the use of a gene therapy/immunotherapy at the time of diagnosis.
    • Expert consultation and ongoing care from pediatric neurologists familiar with early symptoms as well as side effects of brain tumors and their treatments.

  • In-Depth

    What is an anaplastic astrocytoma?

    An anaplastic astrocytoma is a high-grade, or malignant, glioma, a tumor of the glial (supportive) tissue of the brain. The term astrocytoma and glioma are often used interchangeably. Anaplastic astrocytoma and glioblastoma multiforme, another type of astrocytoma, account for approximately 10 percent of pediatric central nervous system tumors.

    As you read further below, you will find general information about anaplastic astrocytomas. If you would like to view summary information about brain tumors first, see the overview on brain tumors.

    Where do anaplastic astrocytomas occur?

    Anaplastic astrocytomas may appear in several areas of the brain. Approximately 65 percent arise in the cerebral hemispheres, the top part of the brain. About 20 percent occur in the area of the thalamus and hypothalamus or the diencephalon, the area responsible for identification of sensation, such as temperature, pain and touch, regulation of appetite/weight and body temperature, as well as connecting the brainstem to the cortex.

    Another 15 percent can occur in the region of the cerebellum and brain stem known as the posterior fossa.

    The median age at diagnosis is 9 to 10 years old, and these tumors occur with equal frequency in boys and girls.

    What causes an anaplastic astrocytoma?

    Most anaplastic astrocytomas and high-grade gliomas occur sporadically or without identifiable cause. They do, however, occur with increased frequency in families with the following conditions.

    • neurofibromatosis type I
    • Li-Fraumeni syndrome
    • hereditary nonpolyposis colon cancer
    • tuberous sclerosis
    • Anaplastic astrocytomas have also been associated with exposure to vinyl chloride and to high doses of radiation therapy to the brain.

    What are the symptoms of an anaplastic astrocytoma?

    Anaplastic astrocytomas of the cerebral hemispheres and the diencephalon (also called supratentorial anaplastic astrocytomas) produce a variety of symptoms as a result of increased pressure within the head, as well as more localizing symptoms related to their specific location, rate of growth and associated inflammation.

    The duration of symptoms varies from slow, subtle onsets to more abrupt presentations. Each child may experience different symptoms. The following are the most common symptoms of an anaplastic astrocytoma:

    • headache and lethargy
    • symptoms of increased pressure within the brain, including:
      • headache (generally upon awakening in the morning).
      • vomiting
      • seizures
    • compression of surrounding brain structures; depending on the location of the tumor, increased pressure can cause:
    • weakness and other motor dysfunction neuroendocrine abnormalities
    • changes in behavior or thought processes

    The symptoms of a brain tumor may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.

    How are anaplastic astrocytomas classified?

    Gliomas, tumors that arise from the glial cells of the brain, are heterogeneous tumors that are classified according to their most aggressive appearing elements. An anaplastic astrocytoma is a type of glioma.

    The World Health Organization classification scheme includes 4 grades of glioma. Anaplastic astrocytoma is a grade III tumor. It originates from the type of glial cell called an astrocyte. It is an aggressive tumor that frequently infiltrates adjacent normal brain tissue and often spreads outside of the central nervous system.

  • Tests

    How is an anaplastic astrocytoma diagnosed?

    Anaplastic astrocytomas are diagnosed using a combination of tests including a: 

    physical examination, including neurologic function tests including: reflexes, muscle strength, eye and mouth movement, coordination and alertness.

    • computerized tomography scan (also called a CT or CAT scan) to capture a which detailed view of the body and to assess the density of tumor tissue versus normal brain. This scan can also tell us if the tumor is creating pressure and causing damage to normal brain tissue taking. 
    • magnetic resonance imaging (MRI) to produce detailed images of organs and structures within the body and/or spine. MRI provides greater anatomical detail than CT scan and can better distinguish between tumor, tumor-related swelling and normal tissue. In addition, MRI can distinguish between a hemorrhage and a tumor cyst.
    • magnetic resonance spectroscopy (MRS), which is done along with MRI to detect the presence of particular organic compounds produced by the body’s metabolism. This also helps us qualify tissue as normal or tumor, and may be able to help us determine where the tumor tissue originated.
    • biopsy to collection of a tissue sample either through a needle or during surgery performed by a pediatric neurosurgeon to confirm the diagnosis.
  • What are the treatments for an anaplastic astrocytoma?

    The primary treatment for newly diagnosed anaplastic astrocytoma is maximal surgical removal, when possible, followed by radiation therapy. To date, no chemotherapy regimen has been demonstrated to increase survival in pediatric high-grade gliomas.

    As with all pediatric cancers, care should be delivered at a specialized center where multidisciplinary teams can provide not only expert diagnostics and experienced medical, surgical and radiation oncologists, but also psychosocial support, neuro-psychological testing and specialized school plans all delivered in a child- and family-sensitive and friendly environment.

    Treatment may include (alone or in combination):

    Surgery

    Surgery has multiple roles in the management of anaplastic astrocytomas, including:

    • treatment of increased intracranial pressure
    • biopsy
    • tumor removal

    For operable tumors, the extent of surgical removal is related to prognosis. Most high-grade gliomas cannot be completely removed because of the way their cells infiltrate other tissue. However, multiple studies have demonstrated that maximal removal results in greater survival.

    Tumors of the cerebral hemispheres are in general more easily removed than those of the midline, diencephalic structures.

    Near complete resection of anaplastic astrocytoma in combination with radiation therapy has been reported to produce a five-year survival rate as high as 40 percent, whereas incompletely removed tumors were associated with a 20 percent five-year survival rate.

    The infiltrating nature of these tumors makes removal difficult.

    Technological advances such as the intra-operative MRI, where surgeons can visualize the tumor as they operate with MRI scans, can enhance efforts at resection of difficult tumors and thereby improve survival.

    Radiation therapy

    Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.

    Chemotherapy

    Chemotherapy is a drug that interferes with the cancer cell's ability to grow or reproduce.

    Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors. Often, a combination of chemotherapy drugs is used. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

    While chemotherapy can effectively treat certain cancers, the drugs do not differentiate normal healthy cells from cancer cells. This means there can be many adverse side effects. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring.

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

    • as a pill to swallow
    • as an injection into the muscle or fat tissue
    • Intravenously, directly to the bloodstream (also called IV)
    • Intrathecally, which means directly delivering the chemotherapy into the spinal column with a needle

    How are side effects managed?

    Side effects in the treatment of anaplastic astrocytoma can arise from surgery, radiation and chemotherapy.

    Procedures should be performed in specialized centers where experienced neurosurgeons, working in the most technologically advanced settings, can provide the most extensive resections while preserving normal brain tissue.

    Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this, inflammation steroids are sometimes necessary.

    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.

    Many specialized brain tumor treatment centers have now 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

    What is the expected outcome after treatment for anaplastic astrocytoma?

    The best reported survival rates for radically removed tumors treated with radiation therapy approaches 40 percent at five years. This is a minority of patients, however, and in most cases, the tumor cannot be completely removed. The five-year survival rates for children whose tumors are incompletely removed remains less than 20 percent. More complete removal of hemispheric tumors may be associated with greater survival.

    What about progressive or recurrent disease?

    Clinical trials and experimental therapies are available for patients with relapsed high-grade gliomas at specialized centers. Current trials include novel medications as well as new methods for the delivery of more traditional agents.

    What is the recommended long-term care for children treated for an anaplastic astrocytoma?

    Children treated for an anaplastic astrocytoma should visit a survivorship clinic every year to:

    • manage disease complications
    • screen for early recurrence of tumor
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    Through the Stop and Shop Family Pediatric Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, children are able to meet with their neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit.

    Endocrinologists, neuro-psychologists, alternative/complementary therapy specialists, and school liaison and psychosocial personnel from the pediatric brain tumor team are also available.

    In addition, children needing rehabilitation may meet with speech, physical, and occupational therapists during and after treatments.

  • Research & Innovation

    What are the latest innovations and research on anaplastic astrocytoma?

    The pediatric neurosurgeons at Children’s Hospital Boston have access to the most recent technological advances such as the intra-operative MRI, which allow them to visualize the tumor as they operate with MRI scans, so they can remove as much of the tumor as possible. 

    We also have access to high-tech imaging, such as PET, CT and functional MRI, which enable us to understand exactly where the tumor tissue is, and to map out surgeries and treatments that minimize risk to healthy brain tissue.

    What is the latest research on anaplastic astrocytoma?

    Clinical and basic scientists at both Children’s Hospital Boston and Dana-Farber Cancer Institute are conducting numerous research studies to help clinicians better understand and treat anaplastic astrocytoma. Dana-Farber/Children’s Hospital Cancer Center is a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed or refractory disease. It is also the New England Phase I Center of the Children’s Oncology Group. Children with progressive/recurrent low-grade glioma of the cerebellum may be eligible for experimental therapies available through these consortiums.

    Our Pediatric Brain Tumor team recently initiated a Phase I gene therapy trial for a pediatric malignant glioma that will evaluate whether the use of AdV-tk followed by anti-herpetic prodrug in combination with radiation therapy will be safe. It will also determine if this immunosuppressive agent can be effectively delivered without disturbing standard therapy and if it will have anti-tumor activity in pediatric patients with malignant glioma.

    For more information on current research, see the Brain Tumor Program.

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