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Cerebellar Pilocytic Astrocytoma

  • What's unique about our program is that we treat every patient differently. We understand the biology that drives tumors, so we are involved in research to develop treatments that are specific to the individual tumor and the individual child."

    - Mark Kieran, MD, PhD, Director, Pediatric Medical Neuro-Oncology

     

    Cerebellar pilocytic astrocytoma is the most common type of low grade glioma, with a very high cure rate of 90 to 95 percent. Low grade gliomas are slow growing, less aggressive cancers of the brain or spine. Children cured of cerebellar pilocytic astrocytoma enjoy a quality of life better than most children who’ve had cancer. A child’s balance, strength and coordination may be compromised if there was damage to the cerebellum, but a child’s fertility and life expectancy should not be affected.

    • The cure rate for cerebellar pilocytic astrocytoma is 90 to 95 percent.
    • The most common treatment is complete surgical removal of the tumor.
    • If the tumor is completely surgically removed, it only reoccurs 5 to 10 percent of the time.
    • Other treatments include chemotherapy and radiation, but usually are not necessary if the tumor is completely removed.
    • Cerebellar pilocytic astrocytoma accounts for about 70 percent of low-grade gliomas.
    • Cerebellar pilocytic astrocytomas are grade I tumors, and cannot turn into grade 2 astrocytoma (fibrillary astrocytoma).
      • Grade refers to the severity of the cancer, and how abnormal the cells are.
    • Coordination and balance are the most common and most noticeable symptomsof cerebellar astrocytomas.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches cerebellar pilocytic astrocytoma

    At Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, we utilize the expertise of both Boston Children's Hospital and Dana-Farber Cancer Institute to treat children and adolescents with all forms of cancer.

    Specialists from Boston Children’s Hospital work closely with the experts at Dana-Farber Cancer Institute to ensure that every aspect of your child’s health is overseen before, during and after the treatment period. Our multidisciplinary approach is used at every step of care, including reading x-rays and MRI scans, interpreting the diagnosis and making clinical decisions.  We can provide your child with expert diagnosis, treatment and care—as well as the benefits of some of the most advanced clinical and scientific research in the world.

    In our Low Grade Glioma Program we take a multidisciplinary approach to researching and treating children with cerebellar pilocytic astrocytoma. Since many of our practicing physicians are also active researchers, your child will get the very best treatment available. Specialists in our program are involved in clinical research for personalized medicine drugs, which target specific parts of the tumor based on the person’s genes.


  • At the Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, our team knows that you’re probably very concerned about your child’s tumor. It might ease your mind to know that this kind of cancer has a very high cure rate and that we’ve developed innovative treatments for children with low grade glioma. Learning more about this condition may help you feel more confident and informed as we work towards healing your child.

    We understand you want to know more about cerebellar pilocytic astrocytoma, and will have many questions including:

    • What is it?
    • What can we do about it?
    • How will it affect my child long-term?

    We’ve provided some answers to these questions here, and when you meet with our experts, we can talk with you more about your child’s diagnosis and treatment.

    What is cerebellar pilocytic astrocytoma?

    A cerebellar pilocytic astrocytoma is a brain tumor arising from a type of cell found in the central nervous system known as a glial cell. These tumors originate from a specific type of glial cell called an astrocyte. Astrocytes make up the supportive network of the brain, providing structural support and several other functions. These cells are named for their star-like appearance. Cerebellar pilocytic astrocytoma grows in the rear part of the brain known as the cerebellum, which controls balance and coordination.

    Cerebellar pilocytic astrocytoma is a grade I tumor, which means it often remains in a small focal area. It is different than grade 2 astrocytoma (fibrillary astrocytoma) which tend to spread out into the surrounding areas. It’s important to note that cerebellar pilocytic astrocytoma does not develop into cerebellar fibrillary astrocytoma.

    How could this tumor affect my child’s mental abilities?

    No. The cerebellum does not control conscious thinking or mental abilities. So pressure on the cerebellum from cerebellum astrocytoma will not affect the child’s mental abilities.

    Causes

    What causes cerebellar pilocytic astrocytoma?

    The cause of cerebellar pilocytic astrocytoma is still under intensive study.  Achromosomal abnormality involving a gene called BRAF, is implicated in a large percentage of these tumors.

    BRAF is a gene that is part of a chain of molecules that receives messages from the surrounding environment of the cell and is partly responsible for  sending messages to tell a cell to divide.

    In cerebellar pilocytic astrocytoma, the molecule BRAF has an error that tells the nucleus to keep dividing. This is caused by a chromosomal abnormality. The uncontrolled division is responsible for cerebellar pilocytic astrocytoma.

    Fortunately, the BRAF mutation is a local mutation. This means that it is an error only present in the tumor cells, and it is not a systemic problem affecting other cells of the body.

    Symptoms

    What are the symptoms of a cerebellar pilocytic astrocytoma?

    Due to the relatively slow growth rate of cerebellar pilocytic astrocytoma, a child with this tumor tend to have symptoms for many months before being seen by a doctor. Some children, however, have a more sudden onset of symptoms and are related to blockage of the flow of cerebral spinal fluid. The following are the most common symptoms of a cerebellar pilocytic astrocytoma, however, each child may experience symptoms differently:

    • More that 90 percent of children have increased pressure within the brain, causing:
      • headache (generally upon awakening in the morning)
      • vomiting
      • fatigue
    • Most affected children have difficulty with balance and coordination.
    • Many children have vision problems, such as seeing double.

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

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

    How is a cerebellar pilocytic astrocytoma diagnosed?

    Diagnostic procedures for a cerebellar pilocytic astrocytoma may include:

    • physical examination - your child may have difficulty walking and coordinating movements of his hands and/or legs
    • 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 (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays. For cerebellar pilocytic astrocytoma, a CT scan of the brain may be done if there is evidence of blockage of spinal fluid.
    • magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets and a computer to produce detailed images of the brain and spine.
    • Biopsy or resection - in many cases, doctors take a tissue sample from the tumor through a needle during a simple surgical procedure. In other cases, the tumor is removed at the time of the initial operative procedure, often to relieve the blockage of spinal fluid flow.
  • We understand how difficult a diagnosis of cerebellar pilocytic astrocytoma can be, both for your child and for your whole family. That's why our team is focused on 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.

    What are the treatments for a cerebellar pilocytic astrocytoma?

    Specific treatment for your child is carefully determined by your child's team based on:

    • 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
    • your opinion or preference

    Treatment may include (alone or in combination):

    • surgery- the initial treatment is usually surgery, and when doctors are able to completely remove the tumor, there is a very high chance of a cure. If the tumor recurs after surgical removal, or if the tumor re-grows after partial surgical removal, the recommended treatment is a second attempt at surgical removal/de-bulking of the tumor. To assist the surgeons, the operations are often performed in the intraoperative MRI, an MRI scanner built inside the operating room to optimize the surgeons ability to remove the tumor safely and completely. 
    • chemotherapy– a drug treatment that works by interfering 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 to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

      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 - chemotherapy given directly into the spinal column with a needl
    • proton radiation therapy- using high-energy particles (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. Radiation is effective at killing cancerous cells, but the side effects can be severe. Since the cure rate from surgery and chemotherapy is so high (around 90 to 95 percent), most children do not need radiation therapy. Severe side effects include:
      • risk for stroke
      • risk of developing new cancer
      • delayed cognitive development

    How are side effects of chemotherapy managed?

    Common side effects include nausea, vomiting and decreased blood counts (anemia). Children take anti-emetics (anti-nausea) medications with the chemotherapy to help with nausea and vomiting.

    Occasionally, children receiving chemotherapy need a transfusion of red blood cells and/or platelets to replace these cells, since chemotherapy temporarily decreases the body's ability to produce red blood cells and platelets. White blood cells are reduced with chemotherapy; however, these cells are not transfused. Occasionally, children take medication to assist their bodies in producing white blood cells.


    Coping & support

    It might be helpful to remember that while cerebellar pilocytic astrocytoma is difficult to deal with, many children and their families have faced similar challenges. There's a lot of support available here at Dana-Farber Children's Hospital for you and your family, and here are some of the ways we can help. Visit our For Patients and Families page for all you need to know about:

    • getting to Dana-Farber/Boston Children's Hospital
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family
  • Research & Innovation

    Dana-Farber/Children's Hospital Cancer Center is involved in clinical trials for developing drugs that target the specific cause of a cancer. Chemotherapy currently cannot differentiate normal cells from cancerous cells, and unfortunately normal cells are often also killed during chemotherapy. These new drugs could identify which cells are cancerous and attack the specific origin of the cancer. Learn more about the research and innovations happening at our Brain Tumor Program.

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