Childhood Glioblastoma Multiforme and Anaplastic Astrocytoma


Information for: Patients | Healthcare Professionals

Glioblastoma Multiforme and Anaplastic Astrocytoma

Overview

Having a tumor in the brain is always a very serious matter, and glioblastoma multiforme and anaplastic astrocytoma are no different. Tumors are masses of abnormal cells that grow out of control. When these tumors originate in the brain, they can be very complicated to treat because of the delicate surrounding tissue.

  • A high-grade glioma is a malignant tumor that arises from the brain’s supportive tissue (glial cells). There are two high-grade gliomas: glioblastoma multiforme (GBM) and anaplastic astrocytomas (AA).
  • GBM and AA come from a type of glial cell called an astrocyte.
  • An astrocytoma is a type of glioma, and sometimes they are referred to as gliomas.
  • These tumors can occur with increased frequency in families with certain genetic diseases, including neurofibromatosis type I (also called NF1), Li-Fraumeni syndrome, hereditary nonpolyposis colon cancer and tuberous sclerosis.
  • GBM and AA are aggressive tumors that rapidly infiltrate adjacent brain tissue and, as a result, they are difficult to treat.

As you read on, you’ll find detailed information about glioblastoma multiforme and anaplastic astrocytoma.

How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches glioblastoma multiforme and anaplastic astrocytoma

Children with high-grade gliomas, such as glioblastoma multiforme or anaplastic astrocytoma, are treated through the Glioma Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital. The Glioma Program is part of the Pediatric Brain Tumor Center at Dana-Farber/Boston Children’s, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors.

Most children diagnosed with glioblastoma multiforme or anaplastic astrocytoma receive surgery and radiation, and in some cases chemotherapy. Our pediatric neuro-oncology and pediatric neurosurgical specialists at Dana-Farber/Boston Children's offer:

  • technological advances, such as the intra-operative MRI, which allow our pediatric neurosurgeons to "see" the tumor as they operate with MRI scans
  • treatment with the best standard of care, including neurosurgery, radiation therapy and chemotherapy
  • access to unique Phase I clinical trials, from our own investigators, Children’s Oncology Group, the Pediatric Oncology Experimental Therapeutics Consortium and the Department of Defense Neurofibromatosis Clinical Trial Consortium

In-depth

We understand how overwhelming a diagnosis of a brain tumor can be. Right now, you probably have a lot of questions. How dangerous is this condition? What is the very best treatment? What do we do next?

We’ve tried to provide some answers to these questions here, and our experts can explain your child’s condition fully when you meet with us.

What is glioblastoma multiforme or anaplastic astrocytoma?

Glioblastoma multiforme (GBM) and anaplastic astrocytomas (AA) are types of brain tumors — masses of tissue that develop from abnormally growing cells. GBM and AA arise from a certain kind of brain cell known as a glial cell - for this reason, they may also be known as "gliomas."

The specific kind of glial cell that they come from is called as an astrocyte, and this is why they can also be called "astrocytomas."  Both GBM and AA are malignant tumors, meaning that they grow and metastasize, or spread. GBM tend to be more aggressive than AA.

What’s the difference between glioblastoma multiforme and anaplastic astrocytomas?

When doctors diagnose a brain tumor, they “stage” it, or give it a grade, according to whether it has spread, and if so, how far. This helps us determine treatment options and prognosis. The World Health Organization classification scheme includes four grades of glioma:

  • Glioblastoma multiforme is a grade IV tumor. This means that they are aggressive tumors that spread to adjacent healthy brain tissue.
  • Anaplastic astrocytoma is a grade III tumor. While anaplastic astrocytomas grow less rapidly than glioblastoma multiforme, they are equally malignant.
Where does glioblastoma multiforme and anaplastic astrocytoma occur?

Glioblastoma multiforme and anaplastic astrocytoma can occur in different parts of the brain. Depending on the size and location of the tumor, children may experience different symptoms. 

  • About 65 percent of these tumors arise in the cerebral hemispheres, which control many higher functions such as speech, movement, thought and sensation.
  • About 20 percent can occur in the area of the thalamus and hypothalamus or the diencephalon, which is responsible for identification of sensation, such as temperature, pain and touch, regulation of appetite/weight and body temperature, and also connects the brainstem to the cortex).
  • Another 15 percent can occur in the region of the cerebellum and brain stem known as the posterior fossa (at the base of the brain). These area coordinate balance and motor function.

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

As you read further below, you’ll find information about glioblastoma multiforme and anaplastic astrocytoma.

What causes a glioblastoma multiforme and anaplastic astrocytoma?

As a parent, you undoubtedly want to know what may have caused your child’s tumor. Unfortunately, doctors don’t have a lot of answers to this question, since high-grade gliomas occur without an identifiable cause in most patients. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

We do know that these tumors can occur with increased frequency in families with certain hereditary conditions, including:

  • Li-Fraumeni syndrome
  • hereditary nonpolyposis colon cancer
  • tuberous sclerosis
  • neurofibromatosis Type 1
What are the symptoms of a glioblastoma multiforme and anaplastic astrocytoma?

Each child may experience symptoms differently and they vary greatly depending on the size and location of the tumor and whether it has spread.

Glioblastoma multiforme and anaplastic astrocytoma can cause symptoms that result from increased pressure within the head, as well as other symptoms related to the tumor’s specific location, rate of growth and associated inflammation.

Symptoms can develop slowly over time or begin very suddenly. The following are the most common:

  • headache and lethargy (generally upon awakening in the morning)
  • seizures, depending on tumor type and location
  • compression of surrounding brain structures. Depending on the location, this can cause:
    • weakness and other motor dysfunction
    • hormonal abnormalities
    • changes in behavior or thought processes
     
How are glioblastoma multiformes and anaplastic astrocytoma classified?

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

Gliomas are composed of different parts and are classified according to their most aggressive appearing elements. The World Health Organization classification scheme includes four grades of glioma.

  • Glioblastoma multiforme is a grade IV tumor.
  • Glioblastoma multiformes are aggressive tumors that spread to adjacent healthy brain tissue.
  • Anaplastic astrocytoma is a grade III tumor.
  • Anaplastic astrocytomas, while less rapidly growing than glioblastoma multiforme, are equally malignant.

Your child’s doctor can provide additional information on the classification of glioblastoma multiforme and anaplastic astrocytoma tumors.

Questions to ask your child’s doctor

After your child is diagnosed with a brain tumor, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.  
 
If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.

Some of the questions you may want to ask include:

  • What type of brain tumor does my child have?
  • Where in the brain is the tumor located? How might this affect my child?
  • Has my child’s brain tumor spread?
  • Can the tumor be treated with surgery?
  • How long will my child need to be in the hospital?
  • What are the possible short and long-term complications of treatment? How will they be addressed?
  • What is the likelihood of cure?
  • What services are available to help my child and my family cope?

FAQ

Q: What is the expected outcome after treatment?

A: Unfortunately, the prognosis for glioblastoma multiforme and anaplastic astrocytoma tumors remains poor. In general, more complete removal of tumors results in a greater chance of survival. Your child’s physician will discuss treatment options with you, including experimental clinical trials and supportive care.

Q: Where will my child be treated?

A: Children treated through Dana-Farber/Boston Children's receive outpatient care at the Jimmy Fund Clinic on the third floor of Dana Farber Cancer Institute. If your child needs to be admitted to the hospital, she will stay at Boston Children's Hospital on the ninth floor of the Berthiaume building.

Q: What services are available to help my child and my family cope?

A: We offer many services to help you, your child and your family get through this difficult time.

Q: What kind of supportive or palliative care is available for my child?

A: When necessary, our Pediatric Advanced Care Team (PACT) is available to provide supportive treatments intended to optimize the quality of life and promote healing and comfort for children with life-threatening illness. In addition, PACT can provide psychosocial support and help arrange end-of-life care when necessary.

Tests

The first step in treating your child is forming an accurate and complete diagnosis.

Your child’s physician may order a number of different tests to determine the type and location of the tumor. Diagnostic procedures for a glioblastoma multiforme and anaplastic astrocytoma are used to determine the exact type of tumor and whether the tumor has spread.

In addition to a physical exam, a medical history and neurological exam (a test of your child’s reflexes, muscle strength, eye and mouth movement, coordination and alertness), diagnostic procedures for glioblastoma multiforme and anaplastic astrocytoma may include:

  • magnetic resonance imaging (MRI) — to produce detailed images of the brain and spine
  • magnetic resonance spectroscopy (MRS) — which is a test done along with MRI at specialized facilities that can detect the presence of particular organic compounds produced by the body's metabolism within sample tissue. This helps us identify tissue as either normal or tumor, and may be able to distinguish between different types of tumors.
  • computerized tomography scan (also called a CT or CAT scan) — to capture a detailed view of the body, and particularly helpful in examining bones and cerebral spinal fluid.
  • biopsy or tissue sample — from the tumor to provide definitive information about the type of tumor. This is collected during surgery.
  • lumbar puncture (spinal tap) — to remove a small sample of cerebrospinal fluid (CSF) and determine if any tumor cells have started to spread into this fluid.

After we complete all necessary tests, our 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.

Treatment and care

We know how difficult a diagnosis of a pediatric brain tumor can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child.

There are a number of treatments we may recommend. 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 primary treatment for newly diagnosed glioblastoma multiforme and anaplastic astrocytoma includes maximal surgical removal, when possible, followed by radiation therapy. To date, no chemotherapy regimen has been demonstrated to increase survival rates in children with pediatric high-grade gliomas.

As with all pediatric cancers, we recommend that care be delivered at specialized centers like our. Here, multidisciplinary teams can provide expert diagnostics and treatment by experienced medical, surgical and radiation oncologists. Also, they can make sure your child has psychosocial support, neuro-psychological testing and specialized school plans.

Treatment may include (alone or in combination):

Surgery

The first treatment is usually neurosurgery to remove as much of the tumor as possible. Our pediatric neurosurgeons are experienced at using advanced techniques, such as intraoperative MRI, to maximize removal of the tumor.

  • Surgery has multiple roles in the management of glioblastoma multiforme and anaplastic astrocytoma, including treatment of increased intracranial pressure, biopsy and tumor removal.
  • Tumor specimens are examined by our pediatric neuropathologists to determine the exact diagnosis.
  • Complete resection or surgical removal of the entire tumor is ideal when possible.
    • Most high-grade gliomas cannot be completely removed because they tend to infiltrate into adjacent healthy tissues.
    • Tumors of the cerebral hemispheres are generally easier to remove than those along the midline of the brain.
     
  • A biopsy of the removed tissue is conducted for diagnostic purposes.
  • In general, the more completely the tumor can be removed, the greater the chances for survival.

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 for difficult tumors and thereby improve survival.

Radiation therapy

Your child may also receive precisely targeted and dosed radiation therapy in order to kill cancer cells left behind after surgery. This is important to control the local growth of tumor, and it helps increase survival in high-grade gliomas.

  • Radiosurgery to deliver additional radiation to residual tumor masses is also being used in specific cases.
  • Other techniques to increase radiation dose have been unable to enhance survival over conventional radiotherapy.
Chemotherapy

Chemotherapy refers to drugs that interferes with the cancer cell’s ability to grow or reproduce. For glioblastoma multiforme and anaplastic astrocytoma, chemotherapy before surgery may help shrink the tumor, making it possible to remove.

  • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
  • Often, we use a combination of chemotherapy drugs.
  • We may give certain chemotherapy drugs in a specific order.

A variety of chemotherapy regimens have been tested in the treatment of newly diagnosed high-grade gliomas.

  • While studies in adults have suggested that certain drugs can produce modest responses in high-grade gliomas, this effect has been less pronounced for pediatric patients.
  • Several regimens have produced responses, but none has improved survival.
  • Increased doses of chemotherapy along with autologous stem cell transplant have also not produced notable advantage.
  • New biologic and immunotherapy-based treatments for newly diagnosed glioblastoma multiforme and anaplastic astrocytoma are now being tested to try and improve the effectiveness of therapy.

Chemotherapy drugs do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. 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, if possible.

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

  • orally, as a pill to swallow
  • intramuscularly, as an injection into the muscle or fat tissue
  • intravenously, directly to the bloodstream
  • intrathecally, directly into the spinal fluid with a needle
How are side effects managed?

Side effects in the treatment of glioblastoma multiforme and 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

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

What is the expected outcome after treatment for glioblastoma multiforme or anaplastic astrocytoma?

Unfortunately, the prognosis for glioblastoma multiforme and anaplastic astrocytoma tumors remains very poor. In general, more complete removal of tumors, when possible, results in a greater chance of survival. Your child’s physician will discuss treatment options with you, including experimental clinical trials, and supportive care.

What about progressive or recurrent disease?

For children with relapsed high-grade gliomas, we offer access to the latest clinical trials and experimental therapies. Current trials include novel medications as well as new methods for the delivery of more traditional agents. Talk to your child’s physician for more information about clinical trials and experimental treatments.

Resources and support

We understand that you may have a lot of questions if your child is diagnosed with a glioblastoma multiforme or an anaplastic astrocytoma. Will it affect my child long-term? What do we do next? We’ve tried to provide some answers to those questions in these pages, but there are also a number of resources and support services to help you and your family through this difficult time.

Innovation and research clinical innovations

The pediatric neurosurgeons at Dana-Farber/Boston Children's 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.

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

For children experiencing seizures, our pediatric neurologists expertly read electroencephalograms (EEGs) to determine the source of seizure activity. They work closely with neurosurgeons to ensure that healthy tissue responsible for everyday functions, such as speech and movement, are minimally damaged during surgery.

What is the latest research on malignant gliomas, including glioblastoma multiforme?

Clinical and basic scientists at Dana-Farber/Boston Children's are conducting numerous research studies to help clinicians better understand and treat malignant gliomas.

We belong to the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with newly diagnosed, relapsed or refractory disease. It is also the New England Phase I Center of the Children’s Oncology Group and the Department of Defense Neurofibromatosis Clinical Trial Consortium.

Through these consortiums, a number of novel therapies are available for children with both newly diagnosed and current brain tumors. Two new protocols include:

  • A Phase II trial of radiation therapy, cetuximab and irinotecan for children with newly diagnosed malignant glioma and diffuse intrinsic pontine glioma.
  • A second trial for newly diagnosed malignant gliomas is a Phase I gene immuno-therapy trial combined with radiation therapy and temozolomide. This combination is toxic to malignant glioma cells and thus stops their growth. More importantly, it can induce an immune response to malignant cells located outside of the tumor's primary site, thus targeting the infiltrative boundary of the tumor that typically results in recurrence.


Learn more about our pediatric Glioma Program.
Learn more about our pediatric brain tumor research and clinical trials.


 
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